"Until There is a Cure... There is the IMF."
Patient Handbook
Published by the International Myeloma Foundation (IMF)
International Myeloma Foundation
12650 Riverside Drive, Suite 206
North Hollywood, CA 91607-3421
Hotline (USA and Canada): 800.452.CURE (2873)
Tel: 818.487.7455
Fax: 818.487.7454
dation
Email: TheIMF@myeloma.org
Website: www.myeloma.org
a
Founa
Dedicated to improving the quality of life of myeloma
patients while working toward prevention and a cure.
yelom
yelom arrow
M MoneB
ofthe
ultipleM ancer
Prepared by Brian G.M. Durie, M.D.
© 2010 International Myeloma Foundation
InternationalM
C
2010/2011 Edition

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taBle of Contents
HANDbook ovERvIEW
1
WHAT IS MYELoMA?
2
bASIC FACTS AboUT MYELoMA
4
WHY MYELoMA HAS To bE TREATED
5
WHAT CAUSES THE MEDICAL PRobLEMS WITH MYELoMA?
6
DIFFERENT TYPES oF MYELoMA
8
STAGING oF MYELoMA
9
TESTING AT DIAGNoSIS
12
TREATMENT oF MYELoMA
14
INITIAL oR FRoNTLINE THERAPY
15
SUPPoRTIvE CARE
18
IF FRoNTLINE THERAPY IS NoT WoRkING
20
QUESTIoNS To ASk YoUR DoCToR
20
TERMS & DEFINITIoNS
23
LISTING oF IMF PUbLICATIoNS
40
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HandBook overview
wHat is MYeloMa?
Multiple myeloma is a bone marrow cancer. Despite periodic media attention, general
Myeloma is literally an "oma," or tumor, involving the "myelo," or blood-producing
public awareness about myeloma is low. The intent of this booklet is to provide basic infor-
cells in the bone marrow. The cel s that are af ected are plasma cel s (a type of white
mation and suggestions about how to cope with this disease.
blood cel ), which are our antibody- (immunoglobulin-) producing cel s. A malignant or
cancerous plasma cel is cal ed a myeloma cel . Myeloma is cal ed "multiple" since there are
The International Myeloma Foundation (IMF) is committed to providing education
frequently multiple patches or areas in bone where tumors or lesions have developed. A
and support for patients and families. This handbook provides a basic understanding of
single lesion is cal ed a solitary plasmacytoma.
myeloma suf icient to al ow patients to make informed decisions about treatment choices.
The handbook is supplemental to the information given by the doctor. Caregivers, family,
Myeloma affects the places where bone marrow is normally active in an adult. This
and friends may also find the information useful.
marrow is in the hol ow area within the bones of the spine, skul , and pelvis, the rib cage,
and the areas around the shoulders and hips. The areas usual y not af ected are the extremi-
Although there is currently no cure for myeloma, it is an eminently treatable disease.
ties: the hands, feet, and lower arm/leg regions. This is very important, since the function of
Many patients go on to lead ful and productive lives for years, even decades, after diagno-
these critical areas is usual y ful y retained.
sis. With increasing research, the overal outlook for patients is improving steadily. knowing
more about the disease, and understanding what can be done to help, reduces anxieties
Myeloma can be discovered at a precancerous stage (see Table 1). In some cases
and makes it easier to come to terms with the diagnosis.
the myeloma cel s build up very slowly in the bone marrow. The very earliest stage is
cal ed MGUS. This is not a cancer, but a condition cal ed Monoclonal Gammopathy of
Myeloma is a very individual disease. Myeloma is often slow moving, but can also
Undetermined Significance. In MGUS, the myeloma cel s constitute fewer than 10% of the
sometimes be much more aggressive. While the doctor assesses each particular situation
bone marrow cel s. The risk of transition from MGUS to active myeloma is very low: only a
and recommends the best approach, the patient plays a central role in helping make these
1% chance each year of fol ow-up. Even if the myeloma cel s are at a higher level of 10­30%
individual treatment decisions. It is important that patients and their families be wel
of the total bone marrow, the growth rate can be very slow and represent indolent/smolder-
informed, ask questions, and give serious thought to alternative strategies or options. A key
ing or asymptomatic myeloma. both MGUS and indolent myeloma can change very slowly
IMF message is "knowledge is Power." knowing about your disease helps you to make the
over a period of years and do not require active treatment. It is very important to establish
best decisions.
the correct diagnosis distinguishing MGUS and indolent myeloma from active or symptom-
Other IMF Information. This booklet focuses on what to do when myeloma is first
atic myeloma, which does require treatment.
discovered. More details about additional therapies such as transplantation, supportive
care, individual drugs, what to do at relapse and concerning clinical trials are available in
other booklets at www.myeloma.org. For any questions or concerns, the IMF Hotline is
always available at 800-452-CURE (2873).
1
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TABLE 1: DEFINITIONS OF MGUS AND MYELOMA
BasiC faCts aBoUt MYeloMa
OLD NAME
NEW NAME
DEFINITION
Although several things appear capable of causing or triggering myeloma, all of the
MGUS
MGUS
· Monoclonal protein present.
details are not known. Things associated with an increased risk of myeloma and related
(Monoclonal
(i.e., no change in name)
· No underlying disease state.
diseases are toxic chemicals (for example, agricultural chemicals and Agent orange used in
Gammopathy of
vietnam as wel as a whole range of petrochemical compounds such as solvents and clean-
Undetermined
Significance)
ing materials), radiation (including atomic radiation), and several viruses including human
immunodeficiency virus (HIv), hepatitis viruses, human herpes virus 8 (HHv-8), HHv-6
SMOLDERING or
ASYMPTOMATIC MYELOMA · Higher level of disease than
and others. There is some family tendency for myeloma: approximately 5% of family mem-
INDOLENT MYELOMA
MGUS, but stil no symptoms
bers are at increased risk of myeloma. Potential screening/early testing can be discussed
or organ damage.*
with your physician.
MYELOMA
SYMPTOMATIC MYELOMA
· Monoclonal protein present, and
Myeloma occurs in adults. The average age of onset of myeloma is in one's early 60s.
· one or more "CRAb" features of
only 5­10% of patients are under the age of 40 years. Myeloma occurs more commonly in
organ damage.*
men and in some racial groups, such as African-Americans.
* oRGAN DAMAGE CLASSIFIED AS "CRAB" or any other significant clinical problem
There are approximately 20,000 new cases of myeloma in the U.S. each year. The
linked to myeloma progression such as recurrent infections or neuropathy
incidence ranges from ~0.5­1 /100,000 among Asians to as high as ~10­12 /100,000
(unrelated to treatment)
among African-American men. At any one time there are over 100,000 myeloma patients
C ­ calcium elevation (>10 mg/L)
undergoing treatment for their disease in the U.S.
R ­ renal dysfunction (creatinine >2 mg/dL)
A ­ anemia (hemoglobin <10 g/dL or >2g/dL decrease)
B ­ bone disease (lytic lesions or osteoporosis)
oNE oR MoRE "CRAB" or other significant problem required for diagnosis
of SYMPToMATIC MYELoMA
3
4
Questions? Call the IMF Hotline:
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wHY MYeloMa Has to Be treated
wHat CaUses tHe MediCal
ProBleMs witH MYeloMa
Myeloma, if left untreated, can cause bone damage, elevated blood calcium, low
blood counts (especially anemia), predisposition to infection, and kidney damage.
Healthy plasma cel s produce immunoglobulins, which are complex proteins that we
because the bones of the spine are often af ected and because myeloma proteins produced
cal "antibodies." Myeloma cel s do not make normal functioning antibodies, but instead
by myeloma cel s can damage nerves, it is common to have spine and nerve problems that
produce a clonal protein, or immunoglobulin, that is known as a "monoclonal protein."
may require urgent attention.
All of the medical problems related to myeloma are caused by the build-up of
In getting treatment for myeloma, it is important to distinguish between urgent prob-
myeloma cells (see Table 2). However, unlike other types of cancer, myeloma can pres-
lems such as bone damage, infection, kidney damage, or nerve pressure, which need
ent patients with many strange complications because myeloma cel s do not just produce
immediate attention, versus overal planning to treat the disease. Sometimes urgent care
tumors; they also release many proteins and other chemicals into the local microenviron-
cannot and should not be delayed. However, early consultation with a hematologist/oncol-
ment of the bone marrow and directly into the blood stream.
ogist familiar with myeloma is encouraged. For example, options of emergency surgery
versus radiation therapy can be discussed. Also, making sure that al treatment options are
· Local effects in the bone marrow. The ef ects in the bone marrow include a reduction
kept open for the future is an important consideration.
in blood cel production and damage to the surrounding bone. The net results are the
many common features of myeloma, such as anemia, predisposition to infection, bone
Once urgent matters have been dealt with, overal plans can be discussed in more
pain, bone fractures, and elevated blood calcium.
detail. Frequently there is time to seek a second opinion or consultation with an expert to
be assured that al options are careful y reviewed. Even if plans seem to be clear, if there
· Effects outside the bone marrow. The ef ects outside the bone marrow are mostly due
are any concerns, questions, or doubts, it is better to have these aired sooner rather than
to the monoclonal protein produced by the myeloma cel s. As the myeloma cel s build
later. Having a mutual y agreed-upon plan with your physician for ongoing treatment is
up in the bone marrow, the immunoglobulin or antibody protein that is specific to the
tremendously important.
myeloma is released into the blood circulation.
This specific immunoglobulin protein or monoclonal protein produced by myeloma cel s
can cause tissue damage at distant sites; for example, kidney damage is not uncommon.
The protein can interfere with blood clotting and/or circulation, and can potential y cause
other organ or tissue damage.
Treatment for myeloma reduces tumor growth as wel as these diverse ef ects from
myeloma proteins and chemicals.
5
6
Questions? Call the IMF Hotline:
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TABLE 2: MEDICAL PROBLEMS RELATED TO MYELOMA
different tYPes of MYeloMa
EffEcts Of IncrEasEd
causE
IMpact On patIEnt
MyElOMa cElls In BOnE
There are dif erent types and subtypes of myeloma. These are based on the type of immuno-
MarrOw
globulin (protein) produced by the myeloma cel . Normal y, the various immunoglobulins
Increase in blood
Release of calcium from damaged · Mental confusion
have dif erent functions in the body. Each immunoglobulin protein is made up of two heavy
calcium (C*)
bone into bloodstream
· Dehydration
· Constipation
chains and two light chains. (See Figure 1). There are five types of heavy protein chains:
· Fatigue
G, A, D, E, and M. There are two types of light protein chains: kappa () and lambda ( or
· Weakness
L). The typing of myeloma (done with a test cal ed "immunofixation" [IFE]) identifies both
· Renal or kidney
the heavy and light chains. Most myeloma patients, about 65%, have IgG (iG) type myeloma
damage (R*)
with or light chains. The next most common type is IgA (iA) type myeloma, also with
Renal problems (R*) ­
Abnormal or monoclonal protein · Sluggish circulation
either or light chains. (See Table 3). IgM, IgD, and IgE myelomas are quite rare.
kidney damage
produced by the myeloma cel s
· Fatigue
is released into the bloodstream
· Mental confusion
FIGURE 1: IMMUNOGLOBULIN STRUCTURE
and can pass into the urine
Light Chain ( or )
(where it is cal ed bence Jones
protein) and produce kidney
damage. High blood calcium,
infections, and other factors can
Heavy Chain (G, A, D or E)
also cause kidney damage.
Anemia (A*)
Decrease in number and activity
· Fatigue
of red blood cel -producing cel s. · Weakness
Bone Damage (B*)
The myeloma cel s activate osteo- · bone pain
· Thinning (osteoporosis) or
clast cel s, which destroy bone,
· bone swel ing
Approximately 30% of patients produce light chains in the urine (such as kappa light
· Areas of more severe damage and block osteoblast cel s, which · Fracture or col apse
cause lytic lesions, fracture, normal y repair damaged bone.
of a bone
chains) as wel as heavy and light chains (such as IgG kappa) in the blood. In about 10% of
or collapse of a vertebra
· Nerve or spinal
patients, the myeloma cel s produce only light chains and no heavy chains. This is cal ed
cord damage
"light chain" or "bence Jones" myeloma. Rarely (in about 1­2% of patients) the myeloma
additional types of organ dysfunction
cel s produce very little or no monoclonal protein of any type. This is cal ed "non-secretory"
myeloma. However, the FreeliteTM test (serum free light chain assay) can detect minute
Abnormal immune function
The myeloma cel s block
· Susceptibility to infection
production of normal antibodies · Delayed recovery from
amounts of light chains in the blood of most of these patients.
against infection
infection
*CRAB criteria: C Calcium; R Renal/kidney; A Anemia; B bone
7
8
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TABLE 3: T YPES OF MYELOMA
TABLE 4: THE DURIE AND SALMON STAGING SYSTEM
HEAVY CHAIN*
LIGHT CHAIN**
MYELOMA TYPE
STAGE
CRITERIA
MEASURED MYELOMA
CELL MASS
IgG:
kappa ()
IgG or IgG immunoglobulin G
(myeloma cells in billions/m2)*
(immunoglobulin G)
or
with kappa or lambda light chains
STAGE I
All of the following:
600 bil ion*
lambda ( or L)
(low cel mass)
· Hemoglobin value >10 g/dL
· Serum calcium value normal
IgA:
kappa ()
IgA or IgA immunoglobulin A
or
with kappa or lambda light chains
or <10.5 mg/dL
(immunoglobulin A)
lambda ( or L)
· bone x-ray, normal bone structure
(scale 0), or solitary bone
* Rarer types are IgD, IgE and IgM. IgM proteins are usually associated
plasmacytoma only
with a different disease called Waldenström's macroglobulinaemia.
· Low M-component production rates
**There are only 2 light chain types
IgG value <5 g/dL;
IgA value <3 g/dL
· Urine light chain M-component on
There are subtle dif erences in the behaviors of dif erent types of myeloma. IgG myeloma
electrophoresis <4 g/24h
has the usual features of myeloma. The IgA type can sometimes be characterized by tumors
outside of the bone. The IgD type can be accompanied by plasma-cel leukemia and more
STAGE II
Fitting neither Stage I nor Stage III
600 to 1,200 bil ion*
(intermediate cel mass)
*myeloma cells in whole body
frequently causes kidney damage. The light chain or bence Jones myelomas are the most
likely to cause kidney damage and/or lead to deposits of light chains in the kidneys and/or
STAGE III
One or more of the following:
>1,200 bil ion*
on nerves or other organs. Depending upon the characteristics of the light chain deposits,
(high cel mass)
· Hemoglobin value <8.5 g/dL
· Serum calcium value >12 mg/dL
this condition is cal ed either amyloid or light chain deposition.
· Advanced lytic bone lesions (scale 3)
· High M-component production rates
staGinG of MYeloMa
IgG value >7 g/dL
IgA value >5 g/dL
· bence Jones protein >12 g/24h
When myeloma is diagnosed, the amount of myeloma in the body varies from patient to
patient. This is cal ed the stage of myeloma. The most commonly used clinical staging sys-
SUBCLASSIFICATION
· A: relatively normal renal function
tem is shown in Table 4, and shows the correlation between the extent of the myeloma and
(either A or b)
(serum creatinine value) <2.0 mg/dL
· b: abnormal renal function
the damage caused, such as bone disease or anemia. The outlook is better when treatment
(serum creatinine value) >2.0 mg/dL
is started early and bone disease or other complications can be prevented. The most com-
Examples: Stage IA (low cell mass
monly used prognostic factor-based staging system is shown in Table 5, and is the result of
with normal renal function);
the col aboration of more than twenty research institutions world-wide.
Stage IIIB (high cell mass with
abnormal renal function)
9
10
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TABLE 5: INTERNATIONAL STAGING SYSTEM (ISS)
testinG at diaGnosis
Staging for Multiple Myeloma
STAGE
VALUES
Table 7 summarizes the typical testing required at the time of diagnosis (baseline testing).
STAGE 1
2M <3.5
TABLE 7: BASELINE TESTING
ALb 3.5
tEst
purpOsE
STAGE 2
2M <3.5
ALb <3.5
Bone marrow biopsy
This is the single most critical test to determine both the
or
Special testing is done
presence and the percentage of myeloma cel s in the bone
2M 3.5 ­ 5.5
to assess prognosis
marrow. In Stage I disease or for a solitary plasmacytoma,
(e.g., chromosomes, immune
direct biopsy of the tumor mass is performed.
typing, staining for amyloid)
STAGE 3
2M >5.5
Chromosome analysis (cytogenetic testing) can reveal good
or poor chromosome features using direct (Giemsa stained
Note: 2M = Serum 2 microglobulin in mg/L
for banding) and/or FISH analysis.
ALb = Serum albumin in g/dL
Blood Testing
Several tests (assessments of so-cal ed prognostic factors, from the Greek words that mean
"knowing ahead") can be used to assess how aggressive the myeloma is in a given patient.
1. Complete blood count
· To assess presence/severity of anemia (low hemoglobin)
In general, higher or abnormal test results indicate more active myeloma, and possibly, less
(CBC)
· To assess for low white cel count
· To assess for low blood platelet count
likelihood of having a long response with treatment (Table 6).
2. Chemistry panel
· Used to assess kidney function (creatinine and BUN),
TABLE 6: PROGNOSTIC FACTORS
liver functions, albumin, calcium level, and LDH
tEst
3. Special protein testing
sIGnIfIcancE
This shows the presence of the monoclonal myeloma
Protein ("spike" protein).
· Serum 2 microglobulin (S 2M)
The higher the level the more advanced the stage
· Serum protein
· The amount of the abnormal myeloma protein.
electrophoresis (SPEP)
· Serum Albumin (S Alb)
The lower the level the higher the stage
· Immunofixation
· Shows the type of myeloma protein [i.e., heavy chain
· C-reative protein (CRP)
Increased with active disease
(G, A, D or E), light chain, kappa (), lambda ( or L)].
· Serum LDH (lactate dehydrogenase)
Increased with active disease
· FREELITE® test
· Can be used to measure the amount of free kappa or
lambda if no SPEP or UPEP abnormality discovered.
· Abnormal chromosomes on bone
Several chromosome deletions or translocations;
marrow cytogenetics and FISH
can be associated with shorter duration of
(Fluorescent In Situ Hybridization)
remission
11
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TABLE 7: BASELINE TESTING CONT'D
treatMent of MYeloMa
tEst
purpOsE
Deciding about treatment is the most important initial decision. As already emphasized,
Urine Testing
Special protein testing similar
Shows the presence, amount, and type of abnormal
baseline testing, staging, and prognostic classification are essential. Treatment is recom-
to serum above:
myeloma protein in urine.
mended for active or symptomatic myeloma. The urgency of treatment depends upon the
· Urine Protein Electophoresis
exact problems faced by an individual patient.
(UPEP)
· Immunofixation
Bone Testing
To assess the presence, severity, and location of any areas
TABLE 8: GOALS OF MYELOMA TREATMENT
of bone damage.
typE Of
OBJEctIVE
EXaMplEs
tIME tO
X-Rays
X-rays are stil the gold standard in searching for myeloma
trEatMEnt
dEcIdE
bone damage. A ful skeletal survey for myeloma using a series
of X-rays is needed to show loss or thinning of bone (osteo-
Stabilizing
Countering the
· Plasmapheresis to thin the blood
Hours to
porosis or osteopenia caused by myeloma bone destruction),
life-threatening
and avoid stroke
Days
lytic lesions, and/or any fracture or col apse of bone.
disruptions to body
· Hemodialysis when kidney
chemistry and the
function is impaired
MRI
Used when X-rays are negative and/or for more detailed test-
immune system
· Drugs to reduce hypercalcemia
ing of particular areas such as spine and/or brain. Can reveal
(may include chemotherapy)
the presence and distribution of disease in the bone marrow
when X-rays show no bone damage. Can also reveal disease
Pal iative
Relieving discomfort
· Radiation to stop bone destruction
outside of bone, which may be pressing on nerves and/or
Days to
and increasing the
· Red cel transfusion or
spinal cord.
Months
patient's ability to
erythropoietin to relieve anemia
function
CT Scan
Used when X-rays are negative and/or for more detailed test-
· Orthopedic surgery to repair
ing of particular areas. Especial y useful for detailed evaluation
and/or strengthen bone
of smal areas of possible bone damage or nerve pressure.
Remission-
Improving symptoms,
· Therapy to kil malignant cel s
Weeks to
Nuclear Medicine Scans
Routine bone scans used for other cancers. Not useful in
Inducing
slowing or arresting the
throughout the body.
Months
myeloma and should not be performed.
course of the disease
· Radiation to kil malignant cel s
at a tumor site
FDG/PET Scan
A much more sensitive Whole Body scanning technique.
or PET/CT Scanning
Useful for disease monitoring, especial y for non-secretory
Curative
Permanent remission*
· Bone marrow transplants as a
Weeks to
disease. CT used to asses sites of PET positive disease.
means of delivering high-dose
Months
chemotherapy
Bone Density Testing
Helpful to assess the severity of diffuse bone loss in
myeloma and to measure the serial improvement with
* Most appropriate definition of cure or permanent remission is under review. Complete response
bisphosphonate therapy.
(including at the molecular level) can be followed by relapse, so long follow-up is required.
13
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initial or frontline tHeraPY
TABLE 9A: FRONTLINE TREATMENT OPTIONS ­ Transplant Eligible
FRONTLINE THERAPY COMMENTS
ADVANTAGES
DISADVANTAGES
It is important for patients to set aside plenty of time to discuss the options with their
VELCADE®*
· Excel ent and approved
· Shows remarkable benefit
· Produces neuropathy that is
option in frontline
· Many combinations available
partial y or completely reversible
hematologist or hematologist/oncologist. In addition to the baseline test results, one must
· Usual y used with
· Preferred in cases of renal
in this setting
dexamethasone
compromise/abnormal
consider:
genetic features
VELCADE®*
· Simplest vELCADE option in
· Excel ent response rates
· Intravenous
IMPORTANT BASELINE QUESTIONS:
with Dexamethasone
frontline therapy
· FDA-approved for frontline
· Potential for side ef ects:
induction
peripheral neuropathy
· Day-to-day functioning ­ is it impacted?
VTD*
· Highly ef ective combination
· very high response rate in
· Intravenous combination
(vELCADE®/
· Ef icacy and side ef ects need
recent phase III trial
· Potential for side ef ects:
· Work ­ will any changes or interruptions be required?
Thalidomide/
physician discussion
· Excel ent outcomes post-
peripheral neuropathy
Dexamethasone)
transplant
· Age ­ is this a factor in treatment selection and expected outcomes?
More Complex
· Many highly ef ective
· Excel ent response rates
· Intravenous combinations
· Treatment side effects ­ how significant will these be?
VELCADE®
combinations
· Some combinations al ow
· Possible increased toxicities
Combinations
· Careful physician discussion
steroid-free treatment
with uncertain benefits
· Other medical issues ­ will they affect treatment choices and
(with Revlimid®,
required regarding combined
agents vs. sequential use of
tolerance to treatment?
Doxil®, or other
agents)
agents over time
· Transplant ­ is high-dose chemotherapy with transplant recommended?
Dexamethasone
· FDA-approved frontline
· An oral approach producing
· Neuropathy and deep vein
plus Thalidomide*
option in the US
remission in 70% of patients
thrombosis (blood clots) are
· Speed of response ­ how rapidly will the treatment work and
· value and side ef ects now
· FDA-approved for frontline
potential concerns
how will that be assessed?
compared to RD or Rd
induction
(see below)
· Initial and later decisions ­ how much needs to be decided on Day 1?
R or RD or Rd
· very ef ective alternative
· Excel ent response rates
· Revlimid® alone can result in less
(RevloDex)*
to Thal/Dex
· oral
ef ective response
It is general y best to keep the door open for stem cel transplantation if you feel it can be
(Revlimid® alone,
· often preferred by both
· General y wel -tolerated and
· Risk of blood clot problems;
with dexamethasone,
physicians and patients
increasingly popular
requires aspirin or another
a future option for you. Although frontline (first therapy after diagnosis) clinical trials are
or Revlimid® with low-
blood thinner
· Possible reduced stem cel harvest
available, you have to be completely comfortable that you might be randomly assigned to
dose dexamethasone)
one treatment versus another. You may become "locked in" to future randomization and
Dexamethasone*
· A simple option for early
· Pulse dexamethasone alone
· Dexamethasone on an intensive
alone
disease management
provides a substantial
schedule can be poorly tolerated
treatments. Make sure you understand the ful scope of the protocol. If one treatment
percentage of the benefit
of the ful vAD
does not work, this does not mean that another treatment cannot work
VAD*
· Prior to novel agents was
· Produces remission
· Needs central line catheter for Iv
extremely well and give an excellent remission.
(vincristine/
induction of choice
in 70% of patients
administration. The catheter can
Adriamycin/
· Now used as a "back-up"
· Doesn't damage normal
trigger infection and blood clot
Dexamethasone)
stem cel s
complications
· Can be basis for
· vincristine can cause nerve damage
stem cel transplant
· New options are available that are
more ef ective and less toxic
* Can be used with or without plan for harvest and transplant.
Further details about treatment options are available in other IMF publications.
To order these, please contact the IMF at 800-452-2873 or visit our website at www.myeloma.org
15
16
Questions? Call the IMF Hotline:
1-800-452-CURE (2873)

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TABLE 9B: FRONTLINE TREATMENT OPTIONS TABLE ­ Transplant Ineligible
sUPPortive Care
FRONTLINE THERAPY
ADVANTAGES
DISADVANTAGES
MP (Melphalan/ Prednisone)
· Taken by mouth
· Can cause bone marrow stem cel
Treatments are available to al eviate the physical and emotional impact of the disease. Early
· Wel tolerated
damage and therefore reduce chances
· Produces excel ent remissions
of successful stem cel transplant
use of supportive care measures is just as important as initiating frontline therapy.
in about 60% of patients
· Ful benefit occurs slowly over several
· Physicians very familiar with
months
TABLE 10: SUPPORTIVE CARE
protocol
· Not ideal if prompt response required
and/or if stem cel transplant planned
SYMPTOM
TREATMENT
COMMENTS
Dexamethasone plus Melphalan
· In combination with melphalan, · The use of melphalan up-front
· blood transfusion (packed red blood cel s:
The treatments are simple, usual y highly
produces more rapid benefit
damages stem cel s
Fatigue and
leukoreduced, virus screened) if anemia severe
beneficial, and improve feeling of wel being.
than MP
· Dexamethasone can be dif icult for
weakness
· Erythropoietin if anemia mild to moderate
older patients. If used, consider 1 day/
due to anemia
week.
Bone Pain
· bisphosphonate
Relief of bone pain is important in itself and
MPT (MP + thalidomide)
· Taken by mouth
· Same as for MP
(e.g., Aredia® 90 mg Iv over 2-4 hrs monthly;
improves physical activity, which in turn
· Wel tolerated
· Thalidomide has risks of neuropathy
Zometa® 4 mg Iv over 15­45 minutes monthly)
promotes bone strength and healing and
· Higher remission rate than MP
and/or blood clot problems (DvT)
· Pain medication as needed (e.g., Tylenol®, oral
improves emotional wel -being. Potential damage
morphine derivatives, Fentanyl® "Pain Patch")
to kidneys and jaws, though rare, can result from
VMP (vELCADE® + MP)
· General y wel tolerated
· Same as for MP
chronic bisphosphonate therapy. Awareness is
· No blood clot risk
· vELCADE® is I.v.
the key to prevention.
· Higher remission rate than MP
· Significant risk of neuropathy
Fever
· Appropriate antibiotics
Although antibiotics should be selected and
MPR (MP + Revlimid®)
· Taken by mouth
· Risk of blood clot problems with
· Neupogen® if necessary to boost low white blood
used with care, it is extremely important that
· Wel tolerated
Revlimid®. Aspirin or another blood
and/or
cel count
infections be brought under control promptly.
· Higher remission rate than MP
thinner required.
evidence
· Intravenous gamma globulin for severe infections
Having an antibiotic on hand for emergency use
of infection
A variety of other therapies are
· Combinations provide a more
· More side ef ects than simpler
· Tests as needed to diagnose the exact type of
(especial y if traveling) is recommended.
sometimes used
infection (except for dangerous biopsies/cultures)
such as Cytoxan®
aggressive approach, if deemed
regimens
should be performed.
(cyclophosphamide) and Etoposide®
necessary
· No added longer-term benefit
(vP-16). Potential combinations include:
· Symptoms of active disease may
· Side ef ects may both reduce quality
Gastrointestinal
· Appropriate medications to treat nausea, vomiting,
Discuss symptoms with healthcare providers;
· vbMCP (M2 protocol)
be control ed more rapidly and
of life and compromise eligibility
side effects
constipation, or diarrhea
severe symptoms may require hospitalization
· vMCP/vbAP (SWoG protocol)
quality of first remission may
for new protocols
· Maintain adequate fluid intake and nutrition
· AbCM (Uk MRC protocol)
be better
Blood clots and
· Clotting events are medical emergencies;
Risk may be reduced by exercise, weight loss,
Further details about treatment options are available in other IMF publications.
thromboembolic
treatment based on event and patient risk factors
not smoking
To order these, please contact the IMF at 800-452-2873 or visit our website at www.myeloma.org
events
· Aspirin or anti-clotting medications may be
prescribed
Peripheral
· Pain medications
Discuss symptoms with healthcare providers.
neuropathy
· Dose adjustment
Early intervention can prevent permanent
· Physical therapy, vitamin
damage and al ow continued treatment.
and other supplements
Do not adjust doses on your own.
Steroid side ef ects · Take with food early in the morning
Report side ef ects and symptoms to healthcare
· be aware of signs and symptoms of infection,
providers. Do not stop or adjust doses on
changes in blood sugar
your own.
· Medications to prevent shingles and yeast infections
Patient education sheets on preventing blood clots and thromboembolic events, managing steroid-associated side effects,
managing myelosuppression, preventing peripheral neuropathy, and managing gastrointestinal side effects are
17
available from the IMF. To order these, please contact the IMF or visit our website at www.myeloma.org
18
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beyond the management of specific symptoms, a whole range of supportive measures is
critical y important:
if frontline tHeraPY is not workinG
· Physical activity ­ Patients should check with their physicians to clarify if full
There are numerous treatment options beyond the scope of this introductory handbook.
physical activity is feasible or if adjustments need to be made because of bone
Emerging new therapies are increasingly available and can provide major benefit.
disease and particular areas of bone damage. Usually, some physical activity such
Please visit the IMF website at www.myeloma.org for more information and regular
as planned walking or swimming, flexibility and strengthening exercises, and/or
updates or cal the IMF at 800-452-CURE (2873).
a personalized yoga program, can be set up.
· Diet ­ No specific diet has been developed for myeloma patients. This is an area
of ongoing research. In general, "healthy diet" recommendations from other
QUestions to ask YoUr doCtor
disease settings such as cardiac disease and cancer in general (e.g., breast cancer)
Treatment decisions are critical y important to the survival and quality of life of the
can be utilized. Caution should be used in two areas:
myeloma patient. To make an informed decision, the patient needs to have the facts. Some
· Vitamin C ­ High doses (i.e., >1000 mg/day) may be counter-productive in
patients want to discuss al aspects of their situation, treatment, and prognosis. others
myeloma and increase the risk of kidney damage.
just want to know what to do next. Most doctors are sensitive to this and wil vary their
· Herbal and vitamin supplements ­ Talk to your doctor or oncology center
approach based on what they perceive to be the patient's wishes.
pharmacist about using supplements along with chemotherapy or other drug
We encourage patients to be explicit about how deeply they want to get into the details of
treatment. Drug interactions can create medical problems. Most pharmacies
the treatment decision. And, no matter how comfortable the patient feels with a doctor, it is
have systems which identify potential interactions with supplements in addition
general y good practice to get more than one opinion before proceeding.
to medications.
· Mental health ­ Your mental health is critical as you move forward with planned
1. Get a complete description of the treatment program:
treatment. Make sure you are comfortable with the treatment planned. Schedule
· What exactly is the treatment?
an appointment with a mental health professional if you believe that you might be
· What are the objectives of the treatment?
depressed, or if others are concerned that you might be depressed.
· Over what period will the treatment be given?
· Regular sleep ­ This is very important for your immune system.
· What is involved? How often must the patient visit a medical facility? Is hospital-
· Make adjustments ­ As much as possible, reduce or eliminate stress in job,
ization required or a probability? What is the likely impact on the patient's ability to
family, or social situations. Avoid contact with school-age children, avoid
function (i.e., work and play)? How do people feel before, during, and after treat-
crowds as much as possible, and wash hands frequently; your immune system
ment? How do they look? What are typical recovery time frames?
is compromised both by the disease and the treatments. Management of the
· What follow-up or maintenance programs are required?
myeloma is the top priority until remission and/or a stable situation has
been achieved.
· What will the treatment program cost? Will it be covered by health insurance?
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2. How well has this treatment worked for others in similar situations?
4. There are always alternatives. You need to ask al of these questions for each of the
Ef ectiveness is measured in many dif erent ways:
alternatives:
· How much experience is there with the treatment? How many patients have
· What are the alternatives to the recommended treatment?
received it? How long have those patients been followed after the treatment?
· What are the relative pros and cons of the alternatives?
· What are the odds of achieving a complete or partial remission? Which factors sug-
· What are the pros and cons of the alternative treatments vs. no treatment?
gest better or worse odds?
because the disease is rare, there are a limited number of practitioners and centers special-
· How long have the patients' remissions lasted? Which factors correlate to long or
izing in myeloma. It is very common for a myeloma patient to seek a second opinion from
to short remissions?
a specialist at a research center while continuing to rely on a local referring physician to
· What would be the options in the event of a relapse? (These options may change
administer and monitor treatment.
in the interim.)
· What are reasonable expectations for relieving symptoms such as bone pain, path-
Making good decisions about treatment requires resourcefulness, careful questioning, seri-
ological fractures, anemia, fatigue, hypercalcemia? What are the factors that predict
ous thought, and courage. but, most of al , it requires that the patient and his/her support
how well these treatments will work for symptoms?
group take charge of the process.
· How long have people who have received the treatment survived? For newer treat-
because there is no known cure, because there are no guarantees, because every individual
ments, how many of the original group of patients are still alive?
is dif erent, the ultimate decision depends on the preferences and priorities of the patient.
3. Side Effects. Like most cancer treatments, myeloma treatments general y use strong
drugs and other measures aimed at destroying malignant cel s and/or rebalancing body
chemistry. Typical y, there are side ef ects. Some manifest themselves during treatment.
others may show up wel after the treatment is completed.
· What side effects have been observed in patients receiving the treatment? When
do they typically occur? In what percentage of patients do they occur? How serious
are the side effects? Are they life threatening? Are they painful? Are they perma-
nent? How long do they last?
· Are there treatments for the side effects? Do the treatments for the side effects
have side effects?
21
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terMs & definitions
antibody: A protein produced by certain white blood cells (plasma cells) to fight infection
and disease in the form of antigens such as bacteria, viruses, toxins, or tumors. Each antibody
can bind only to a specific antigen. The purpose of this binding is to help destroy the antigen.
accrual: The process of enrolling patients in a clinical research study (trial), or the number of
Antibodies can work in several ways, depending on the nature of the antigen. Some antibod-
patients already enrolled in a trial or anticipated to enroll in a trial.
ies disable antigens directly. Others make the antigen more vulnerable to destruction by other
white blood cells.
acute: A sudden onset of symptoms or disease.
anti-emetic agent: A drug that prevents or controls nausea and vomiting.
albumin: Simple water-soluble proteins that are found in blood serum and many other ani-
mal and plant tissues.
antifungal agent: A drug used to treat fungal infections.
antigen:
alkylating agent: A chemotherapeutic agent such as melphalan or cyclophosphamide.
Any foreign substance (such as a bacteria, virus, toxin or tumor) that, when intro-
Alkylating refers to the way in which these agents cross-link the DNA of myeloma cells and
duced into or arising in the body, causes the immune system to produce natural antibodies.
block cell division.
antineoplastic agent: A drug that prevents, kills, or blocks the growth and spread of cancer
allogeneic: See "Transplantation."
cells.
appendicular skeleton:
amyloidosis: A condition in which myeloma light chains (Bence Jones proteins) are deposited
The long bones (i.e., arms and legs), which are attached to spine,
in tissues and organs throughout the body. This occurs more commonly with lambda versus
chest and pelvis.
kappa Bence Jones proteins. In patients with amyloidosis, the light chain proteins bind to
apoptosis: A normal cellular process involving a genetically programmed series of events lead-
certain tissues such as heart, nerves and kidney rather than being excreted out of the body
ing to the death of a cell.
through the kidneys.
aspiration: The process of removing fluid or tissue, or both, from a specific area.
analgesic: Any drug that relieves pain. Aspirin and acetaminophen are mild analgesics.
asymptomatic myeloma: Myeloma that presents no signs or symptoms of disease. Also called
analog: A chemical compound that is structurally similar to another but differs slightly in
indolent, smoldering, or early myeloma.
composition.
axial skeleton: The skull, spine, and pelvis region of the skeleton.
anemia: A decrease in the hemoglobin, usually below 10 g/dL with over 13-14 g/dL being
normal. Myeloma in the bone marrow blocks red blood cell production, causing shortness of
B cells: White blood cells that develop into plasma cells in the bone marrow and are the source
breath, weakness, and tiredness.
of antibodies. Also known as B lymphocytes.
anesthesia: Loss of feeling or awareness. Local anesthesia causes loss of feeling in a part of the
Basophil: A type of white blood cell. Basophils are granulocytes.
body. General anesthesia puts the person to sleep.
Bence Jones: A myeloma monoclonal protein present in urine. The amount of Bence Jones
angiogenesis: Blood vessel formation, which usually accompanies the growth of malignant
protein is expressed in terms of grams per 24 hours. Normally a very small amount of protein
tissue, including myeloma.
(<0.1 g/24 h) can be present in the urine, but this is albumin rather than Bence Jones protein.
The presence of any Bence Jones protein is abnormal.
angiogenesis inhibitors: Compounds that attempt to cut off the blood supply to tumors.
antibiotics: Drugs used to treat infection.
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Benign: Not cancerous; does not invade nearby tissue or spread to other parts of the body.
Calcium: A mineral found mainly in the hard part of bone matrix or hydroxyapatite.
MGUS is a benign condition.
Cancer: A term for diseases in which malignant cells divide without control. Cancer cells can
Beta 2 microglobulin (2M): A small protein found in the blood. High levels occur in
invade nearby tissues and spread through the bloodstream and lymphatic system to other parts
patients with active myeloma. Low or normal levels occur in patients with early myeloma and/
of the body.
or inactive disease. Approximately 10% of patients have myeloma that does not produce 2M.
At the time of relapse, 2M can increase before there is any change in the myeloma protein
Carcinogen: Any substance or agent that produces or stimulates cancer growth.
level. Factors such as viral infection can sometimes produce elevated serum 2M levels.
Cat or Ct [Computerized (axial) tomography scan]: A test using computerized X-rays to
Biopsy: The removal of a sample of tissue for microscopic examination to aid in diagnosis.
create three-dimensional images of organs and structures inside the body, used to detect small
areas of bone damage or soft tissue involvement.
Bisphosphonate: A type of drug that binds to the surface of bone where it is being resorbed
(or destroyed) and protects against osteoclast activity.
Catheter: A tube that is placed in a blood vessel to provide a pathway for drugs or nutrients.
A Central Venous Catheter is a special tubing that is surgically inserted into a large vein near
Blood cells: Minute structures produced in the bone marrow; they include red blood cells,
the heart and exits from the chest or abdomen. The catheter allows medications, fluids, or
white blood cells, and platelets.
blood products to be given and blood samples to be taken.
Blood count: The number of red blood cells, white blood cells, and platelets in a sample of
Cell: The basic unit of any living organism.
blood.
Cell differentiation: The process during which young, immature (unspecialized) cells take on
Bone marrow: The soft, spongy tissue in the center of bones that produces white blood cells,
individual characteristics and reach their mature (specialized) form and function.
red blood cells, and platelets.
Cell proliferation: An increase in the number of cells as a result of cell growth and cell
Bone marrow aspiration: The removal, by a needle, of a sample of fluid and cells from the
division.
bone marrow for examination under a microscope.
Chemotherapy: The treatment of cancer with drugs that kill all rapidly-dividing cells.
Bone marrow biopsy: The removal, by a needle, of a sample of tissue from the bone. The
cells are checked to see whether they are cancerous. If cancerous plasma cells are found, the
· Combination chemotherapy ­ The use of more than one drug given in a chemotherapy
pathologist estimates how much of the bone marrow is affected. Bone marrow biopsy is usu-
regimen during cancer treatment.
ally done at the same time as bone marrow aspiration.
Chromosome: A strand of DNA and proteins in the nucleus of a cell. Chromosomes carry
genes and function in the transmission of genetic information. Normally, human cells
Bone remodeling: The normal coordination (coupling) between osteoclast cells (which resorb
contain 46 chromosomes.
or destroy bone) and osteoblast cells (which create new bone matrix) to maintain a balanced
state of bone production and destruction.
Chronic: Persisting over a long period of time.
BUn (Blood Urea nitrogen): A measure of the urea level in the blood. Urea is cleared by the
Clinical: Involving direct observation of a patient.
kidney. BUN is a laboratory blood test to assess how well the kidney is functioning. Diseases,
such as myeloma, which compromise kidney function, frequently lead to increased levels of
BUN.
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Clinical trial: A research study of new treatment that involves patients. Each study is
Creatinine: A small chemical compound normally excreted by the kidneys. If the kidneys are
designed to find better ways to prevent, detect, diagnose, or treat cancer and to answer
damaged, the serum level of creatinine builds up, resulting in an elevated serum creatinine.
scientific questions.
The serum creatinine test is used to measure kidney function.
· Control group ­ The arm of a randomized clinical trial that gets the standard treatment.
Cyst: An accumulation of fluid or semi-solid material within a sac.
· End Point ­ What a clinical trial is trying to measure or find out; the goal of the trial.
Typical end points include measurements of toxicity, response rate, and survival.
Cytokine: A substance secreted by cells of the immune system that stimulates growth/activ-
ity in a particular type of cell. Cytokines are produced locally (i.e., in the bone marrow) and
· Experimental group ­ The arm of a randomized trial that gets the new treatment.
circulate in the bloodstream.
· Randomized clinical trial ­ A research study in which subjects are randomly assigned to
receive a particular treatment.
deXa (dual Photon X-ray absorptiometry) study: Measures the amount of bone loss; the
best measure of bone density.
· Phase I trial ­ A trial designed to determine the MTD (maximum tolerated dose) of a
new drug or a new combination of drugs that has never been tried in humans. It is usu-
dexamethasone: A powerful corticosteroid given alone or with other drugs.
ally the first human testing of a new treatment, although in phase I trials of combination
therapies, the individual elements may already have been well tested. Patients in phase I
diagnosis: The process of identifying a disease by its signs and symptoms.
trials must have advanced cancer that is refractory to any standard treatment. In a typical
phase I trial, successive groups ("cohorts") of 3 to 6 patients are given the treatment. All
dialysis: When a patient's kidneys are unable to filter blood, the blood is cleaned by passing
patients in a cohort get the same dose. The first cohort typically gets a very low dose, and
it through a dialysis machine.
the dose is raised in each subsequent cohort until a set number of patients experience DLT
(dose limiting toxicity). The dose level used for the previous cohort is then taken to be the
disease-free survival: The length of time the patient survives without any detectable cancer.
Maximum Tolerated Dose. This dose is then used in a phase II trial.
dlt (dose limiting toxicity): Side-effects that are severe enough to prevent giving more
· Phase II trial ­ A trial designed to determine the response rate of a new therapy that has
of the treatment.
already been tested in phase I trials. Typically, 14 to 50 patients with one type of cancer are
treated to see how many have a response. Patients are usually required to have advanced
dna: The substance of heredity; a large molecule that carries the genetic information that
cancer that is refractory to any standard treatment, and in addition, they must have
cells need to replicate and to produce proteins.
measurable disease. If results from a phase II trial are promising enough, the treatment
may then be tested in a phase III trial. If the results are obviously much better than the
drug resistance: The result of cells' ability to resist the effects of a specific drug.
standard treatment, then it may not be necessary to do a phase III trial, and the treatment
may become standard based on phase II trial results.
edema: Swelling; an abnormal accumulation of fluid in part of the body.
· Phase III trial ­ A trial designed to compare two or more treatments for a given type
efficacy: The power to produce an effect; in cancer research `efficacy' refers to whether the
and stage of cancer. The end point of a phase III trial is usually survival or disease-free
treatment is effective.
survival. Phase III trials are usually randomized, so patients don't choose which treatment
they receive. A typical phase III trial has 50 to thousands of patients. Some phase III trials
electrophoresis: A laboratory test in which a patient's serum (blood) or urine molecules are
compare a new treatment that has had good results in phase II trials with an older, well
subjected to separation according to their size and electrical charge. For myeloma patients,
known, standard treatment. Other phase III trials compare treatments that are already
electrophoresis of the blood or urine allows both the calculation of the amount of myeloma
in common use. Some treatments in phase III trials may be available outside the clinical
protein (M-protein) as well as the identification of the specific M-spike characteristic for each
trial setting.
patient. Electrophoresis is used as a tool both for diagnosis and for monitoring.
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enzyme: A substance that affects the rate at which chemical changes take place in the body.
Herpes simplex: A common virus, it causes sores often seen around the mouth, commonly
called cold sores.
erythrocytes: Red blood cells (RBCs). RBCs carry oxygen to body cells and carbon dioxide
away from body cells.
Herpes zoster: A virus that settles around certain nerves in patients who have previously had
a chicken pox (varicella) infection, causing blisters, swelling, and pain. This condition is also
erythropoietin: A hormone produced by the kidneys. Myeloma patients with damaged kid-
called shingles.
neys don't produce enough erythropoietin and can become anemic. Injections with synthetic
erythropoietin can be helpful. Blood transfusion is another alternative, especially in an emer-
Hormones: Chemicals produced by various glands of the body that regulate the actions of
gency. Synthetic erythropoietin is used as a supportive therapy during anti-myeloma treatment
certain cells or organs.
to avoid anemia.
Human leukocyte antigen (Hla) test: A blood test used to match a blood or bone marrow
free light chains: A portion of the monoclonal protein of light molecular weight that can be
donor to a recipient for transfusion or transplant.
measured in a sensitive assay, the Freelite® test.
Hypercalcemia: A higher-than-normal level of calcium in the blood. This condition can cause
Gene: A specific sequence of DNA or RNA; the biological unit of heredity located in a specific
a number of symptoms, including loss of appetite, nausea, thirst, fatigue, muscle weakness,
place on a chromosome and found in all cells in the body. When genes are missing or dam-
restlessness, and confusion. Common in myeloma patients and usually resulting from bone
aged, cancer may occur.
destruction with release of calcium into the blood stream. Often associated with reduced
kidney function since calcium can be toxic to the kidneys. For this reason, hypercalcemia is
Gene therapy: Treatment that alters genes. Using genes to stimulate the immune system. In
usually treated on an emergency basis using IV fluids combined with drugs to reduce bone
studies of gene therapy for cancer, researchers are trying to improve the body's natural ability
destruction along with direct treatment for the myeloma.
to fight the disease and to make the tumor more sensitive to other kinds of therapy. Treatment
focuses on replacing damaged or missing genes with healthy copies.
igG, iga: The two most common types of myeloma. The G and the A refer to the type of
protein produced by the myeloma cells. The myeloma protein, which is an immunoglobulin,
Genetic: Inherited; having to do with information that is passed from parents to children
consists of two heavy chains, (for example of a G type) combined with two light chains, which
through DNA in the genes.
are either kappa or lambda. Therefore, the two most common subtypes of myeloma have iden-
tical heavy chains (i.e. IgG kappa and IgG lambda). The terms heavy and light refer to the size
Graft-versus-host disease (GvHd): A reaction of donated bone marrow against the recipient's
or molecular weight of the protein, with the heavy chains being larger than the light chains.
own tissue.
Since the light chains are smaller, they are more likely to leak out into the urine, resulting in
urine Bence Jones protein.
Granulocyte: A type of white blood cell that kills bacteria. Neutrophils, eosinophils, and
basophils are granulocytes.
igd, ige: Two types of myeloma that occur less frequently.
Hematocrit (Hct): The percentage of red blood cells in the blood. A low hematocrit measure-
igM: Usually associated with Waldenstrom's macroglobulemia. In rare cases can be a type of
ment indicates anemia.
myeloma.
Hematologic: Originating in the blood, or disseminated by the circulation or through the
immune system: The complex group of organs and cells that produces antibodies to defend
bloodstream.
the body against foreign substances such as bacteria, viruses, toxins, and cancers.
Hematologist: A doctor who specializes in the problems of blood and bone marrow.
immunodeficiency: A lowering of the body's ability to fight off infection and disease.
Hemoglobin: A protein in red blood cells which carries oxygen in the blood.
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immunofixation: An immunologic test of the serum or urine used to identify proteins in the
interleukin: A naturally produced chemical released by the body or a substance used in bio-
blood. For myeloma patients, it enables the doctor to identify the M-protein type (IgG, IgA,
logical therapy. Interleukins stimulate the growth and activities of certain kinds of white blood
kappa, or lambda). The most sensitive routine immunostaining technique, it identifies the
cells. Interleukin-2 (IL-2) is a type of biological response modifier that stimulates the growth
exact heavy and light chain type of M-protein.
of certain blood cells in the immune system that can fight some types of cancer. Interleukin-6
(IL-6) is a cytokine which is a potent stimulus to osteoclast and plasma cell activities.
immunoglobulin (ig): A protein produced by plasma cells; an essential part of the body's
immune system. Immunoglobulins attach to foreign substances (antigens) and assist in
ldH: Lactate dehydrogenase, an enzyme that may be used to monitor myeloma activity.
destroying them. The classes of immunoglobulins are IgA, IgG, IgM, IgD, and IgE.
lesion: An area of abnormal tissue change. A lump or abscess that may be caused by injury
immunosuppression: Weakening of the immune system that causes a lowered ability to fight
or disease, such as cancer. In myeloma, "lesion" can refer to a plasmacytoma or a hole in the
infection and disease. Immunosuppression may be deliberate, such as in preparation for bone
bone.
marrow transplantation to prevent rejection by the host of the donor tissue, or incidental, such
as often results from chemotherapy for the treatment of cancer.
leukocytes: Cells that help the body fight infections and other diseases. Also called white
blood cells (WBCs).
immunotherapy: Treatment that stimulates the body's natural defenses to fight cancer. Also
called biological therapy.
leukopenia: A low number of white blood cells.
incidence: The number of new cases of a disease diagnosed each year.
lymphocytes: White blood cells that fight infection and disease.
lytic lesions:
induction therapy: The initial treatment used in an effort to achieve remission in a newly
The damaged area of a bone that shows up as a dark spot on an X-ray when
diagnosed myeloma patient.
enough of the healthy bone in any one area is eaten away. Lytic lesions look like holes in the
bone and are evidence that the bone is being weakened.
informed consent: The process requiring a doctor to give a patient enough information about
M proteins (M spike): Antibodies or parts of antibodies found in unusually large amounts
a proposed procedure for the patient to make an informed decision about whether or not to
in the blood or urine of multiple myeloma patients. M spike refers to the sharp pattern that
undergo it. The doctor must, in addition to explaining all procedures, address the issues of
occurs on protein electrophoresis when an M protein is present. Synonymous with monoclo-
risks, benefits, alternatives, and potential costs.
nal protein and myeloma protein. (see "monoclonal" below)
infusion: Delivering fluids or medications into the bloodstream over a period of time.
Maintenance therapy: Drugs given to patients in remission to delay or prevent a relapse.
infusion pump: A device that delivers measured amounts of fluids or medications into the
Malignant: Cancerous; capable of invading nearby tissue and spreading to other parts of the
bloodstream over a period of time.
body.
inhibit: To stop something, to hold in check.
Mdr (Multi drug resistance): A resistance to standard treatment, typically associated with
resistance to Adriamycin and vincristine, both chemotherapy drugs. The resistance is caused
injection: Pushing a medication into the body with the use of a syringe and needle.
by a buildup of the p-glycoprotein in the outer cell membrane of the myeloma cells. This
results in drugs being kicked back out of the myeloma cell instead of building up and eventu-
interferon: A naturally produced hormone (cytokine) released by the body in response to
ally killing that cell.
infection or disease which stimulates the growth of certain disease-fighting blood cells in the
immune system. Interferon can be artificially produced by genetic engineering techniques and
Melanoma: A cancer of the pigment-forming cells of the skin or the retina of the eye. Not
used as a form of immunotherapy, primarily in the maintenance (plateau) phase to block any
associated with myeloma despite the similar-sounding name.
regrowth of myeloma and thus delay or prevent relapse.
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Metastasize: To spread from one part of the body to another. When cancer cells metastasize
neoplasm: A new growth of tissue or cells; a tumor that can be referred to as benign or
and form secondary tumors, the cells in the metastatic tumor are like those in the original
malignant.
(primary) tumor. This term is commonly used to describe a disease process in solid tumors
(e.g., breast, prostate) and not in myeloma, which is a blood-related cancer.
neutropenia: A reduced level of neutrophils. Cytotoxic chemotherapy has a tendency to
induce neutropenia. In contrast, lymphocytes which are more important in viral infections,
MGUs (Monoclonal Gammopathy of Undetermined significance): A benign condition in
tend not to be affected by cytotoxic treatment. Neutropenia can be prevented or reduced
which the M protein is present but there is no underlying disease.
using a synthetic hormone called G-CSF (e.g. Neupogen).
Molecule: The smallest particle of a substance that retains all the properties of the substance
neutrophil: A type of white blood cell necessary to combat bacterial infection.
and is composed of one or more atoms.
oncogene: A gene or DNA sequence that normally directs cell growth, but which can also
Monoclonal: A clone or duplicate of a single cell. Myeloma develops from a single malignant
promote or allow the uncontrolled growth of cancer if damaged (mutated) by an environmen-
plasma cell (monoclone). The type of myeloma protein produced is also monoclonal; a single
tal exposure to carcinogens, or if damaged or missing because of an inherited defect. A gene
form rather than many forms (polyclonal). The important practical aspect of a monoclonal
that has the potential to cause a normal cell to become cancerous.
protein is that it shows up as a sharp spike (M spike) in the serum electrophoresis test.
oncologist: A doctor who specializes in treating cancer. Some oncologists specialize in a
Monoclonal antibodies: Artificially manufactured antibodies specifically designed to find and
particular type of cancer treatment.
bind to cancer cells for diagnostic or treatment purposes. They can be used alone, or they can
be used to deliver drugs, toxins, or radioactive material directly to tumor cells.
osteoblast: The cell that produces osteoid, which becomes mineralized with calcium to form
new hard bone.
Monocyte: A type of white blood cell.
osteoclast: A cell found in the bone marrow at the junction between the bone marrow and
Mri (Magnetic resonance imaging): A diagnostic test that uses magnetic energy, rather than
the bone that resorbs or breaks down old bone. In myeloma, the osteoclasts are over-stimu-
X-ray energy, to produce detailed two- or three-dimensional images of organs and structures
lated while osteoblast activity is blocked. The combination of accelerated bone resorption and
inside the body. Gives very fine resolution of soft tissues, especially encroachments on the
blocked new bone formation results in lytic lesions.
spinal cord, but is less accurate for bone lesions.
osteoid: The protein product which becomes mineralized with calcium to form hard bones.
Mtd (Maximum tolerated dose): The highest dose of a treatment that most people can
safely withstand.
osteonecrosis of the jaw: A previously rare jaw problem now being observed in a small per-
centage of patients taking bisphosphonates. The condition produces pain, swelling, and bone
Myelodysplastic syndrome: A condition in which the bone marrow does not function nor-
damage around the tooth sockets in the jaws. There is bone necrosis or loss of bone which can
mally and does not produce enough blood cells. This condition may progress and become
lead to loose teeth, sharp edges of exposed bone, bone spurs, and the breaking loose of small
acute leukemia.
bone spicules or dead bone. A case definition is 3 months with non-healing exposed bone.
Symptoms may not be obvious at first, or may include pain, swelling, numbness or a "heavy
Myeloid: Referring to myelocytes, a type of white blood cell. Also called myelogenous.
jaw" feeling, or loosening of a tooth.
Multiple myeloma is a non-myeloid cancer.
osteoporosis: Reduction in bone density typically associated with old age. Diffuse involve-
Myelosuppression: A decrease in the production of red blood cells, platelets, and some white
ment of bones with myeloma produces what looks like osteoporosis on X-ray and bone
blood cells by the bone marrow.
density measurement.
neoplasia: Abnormal new growth of cells.
Palliative treatment: Aimed to improve the quality of life by relieving pain and symptoms of
disease but not intended to alter its course.
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Pathological fracture: A break in a bone usually caused by cancer or some disease condition.
Prognosis: The projected outcome or course of a disease; the chance of recovery; the life
Occurs in myeloma-weakened bones, which can't bear normal weight or stress.
expectancy.
Pathology: The study of disease by the examination of tissues and body fluids under the
Progression-free survival: The time period during which the patient survives and the cancer
microscope. A doctor who specializes in pathology is called a pathologist.
does not become worse. The improved survival of a patient that can be directly attributed to
the treatment given for the myeloma. This term identifies myeloma patients who are in com-
Pet (Positron emission tomography) scan: A diagnostic test that uses a sophisticated cam-
plete remission versus those who have had an episode of relapse or progression.
era and computer to produce images of the body. PET scans show the difference between
healthy and abnormally functioning tissues.
Progressive disease: Disease that is becoming worse, as documented by tests.
Placebo: An inert (inactive) substance often used in clinical trials for comparison with an
Protocol: A detailed plan of treatment including the dose and schedule of any drugs used.
experimental drug.
Precancerous: A term used to describe a condition that may, or is likely to become, cancer.
Plasma: The liquid part of the blood in which red blood cells, white blood cells, and platelets
are suspended.
radiation therapy: Treatment with x-rays, gamma rays, or electrons to damage or kill malig-
nant cells. The radiation may come from outside the body (external radiation) or from radio-
Plasma cells: Special white blood cells that produce antibodies. The malignant cell in myelo-
active materials placed directly in the tumor (implant radiation).
ma. Normal plasma cells produce antibodies to fight infection. In myeloma, malignant plasma
cells produce large amounts of abnormal antibodies that lack the capability to fight infection.
radiologist: A doctor who specializes in creating and interpreting images of areas inside the
The abnormal antibodies are the monoclonal protein, or M protein. Plasma cells also produce
body. The images are produced with x-rays, sound waves, magnetic fields, or other types
other chemicals that can cause organ and tissue damage (i.e., anemia, kidney damage, and
of energy.
nerve damage).
recurrence: The reappearance of a disease after a period of remission.
Plasmacytoma: A collection of plasma cells found in a single location rather than diffusely
throughout the bone marrow, soft tissue, or bone.
red blood cells (erythrocytes): Cells in the blood that contain hemoglobin and deliver oxy-
gen to and take carbon dioxide from all parts of the body. Red cell production is stimulated
Plasmapheresis: The process of removing certain proteins from the blood. Plasmapheresis can
by a hormone (erythropoietin) produced by the kidneys. Myeloma patients with damaged
be used to remove high levels of monoclonal myeloma protein from the blood of multiple
kidneys don't produce enough erythropoietin and can become anemic. Injections with
myeloma patients.
synthetic erythropoietin can be helpful. Blood transfusion is another alternative, especially
in an emergency. Synthetic erythropoietin is a supportive therapy used during anti-myeloma
Platelet: One of the three major blood elements, others being the red blood cells and white
treatment to avoid anemia.
blood cells. Platelets plug up breaks in the blood vessel walls and release substances that
stimulate blood clot formation. Platelets are the major defense against bleeding. Also called
refractory: Disease that is unresponsive to standard treatments.
thrombocytes.
regression: The shrinkage of cancer growth.
Port ­ implanted: A catheter connected to a quarter-sized disc that is surgically placed just
below the skin in the chest or abdomen. The catheter is inserted into a large vein or artery
relapse: The reappearance of signs and symptoms of a disease after a period of improvement.
directly into the bloodstream. Fluids, drugs, or blood products can be infused, and blood can
be drawn through a needle that is stuck into the disc.
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remission or response: Complete or partial disappearance of the signs and symptoms of
steroid: A type of hormone. Steroids are often given to patients along with one or more anti-
cancer. Remission and response are used interchangeably.
cancer drugs and appear to help to control the effects of the disease on the body.
· Complete Remission (CR) ­ CR is the absence of myeloma protein from the serum and/or
urine by standard testing; absence of myeloma cells from the bone marrow and/ or other
supportive care: Treatment given to prevent, control, or relieve complications and side effects
areas of myeloma involvement; clinical remission and improvement of other laboratory
and to improve the patient's comfort and quality of life.
parameters to normal. CR is not the same thing as a cure.
systemic therapy: Treatment using substances that travel through the bloodstream, reaching
· Very Good Partial Remission (VGPR) ­ VGPR is just less than CR, that is, when myeloma
and affecting cancer cells all over the body.
protein levels are reduced by 90%, but not gone.
thrombocytes: See "Platelets."
· Partial Remission (PR) ­ PR is a level of response less than CR. In SWOG studies, it has
meant >50% and <75% response. In other studies it has meant >50% response.
thrombocytopenia: A low number of platelets in the blood. The normal level is 150,000-
rna (ribonucleic acid): Any of various nucleic acids that are associated with the control of
250,000. If the platelet count is less than 50,000, bleeding problems could occur. Major
cellular chemical activities. RNA is one of the two nucleic acids found in all cells ­ the other
bleeding is usually associated with a reduction to less than 10,000.
is DNA (deoxyribonucleic acid). RNA transfers genetic information from DNA to proteins
produced by the cell.
tnf (tumor necrosis factor): A type of biological response modifier that can improve the
body's natural response to disease.
serum osteocalcin: A protein produced and secreted by osteoblasts when they are making
osteoid. A low level reflects active myeloma. A higher than normal level reflects more stable
toxins: Poisons produced by certain animals, plants, or bacteria.
myeloma.
transfusion: The transfer of blood or blood products.
shingles: See "Herpes zoster."
transplantation: There are several different types of transplantation.
side effects: Problems that occur due to drugs used for disease treatment. Common side
· Bone marrow transplantation ­ This term refers to the process of collecting stem cells
effects of cancer chemotherapy are fatigue, nausea, vomiting, decreased blood cell counts, hair
from the bone marrow and infusing them into a patient. This term is used less frequently
loss, and mouth sores.
today in myeloma as stem cells are now collected from the peripheral or circulating
blood.
skeletal survey (metastatic survey): A series of plain X-rays of the skull, spine, ribs, pelvis, and
long bones to look for lytic lesions and/or osteoporosis.
· Peripheral blood stem cell transplantation ­ Doctors remove healthy stem cells from a
patient's circulating blood system (not from the bone marrow) and store them before
stable disease: This describes patients who have some response to treatment but
the patient receives high-dose chemotherapy to destroy the cancer cells. The stem cells
<50% reduction in myeloma protein levels. Stable disease is not necessarily bad or sub-optimal
are then returned to the patient, where they can produce new blood cells to replace cells
(as compared with CR or PR) provided the myeloma has stabilized and is not progressing.
destroyed by the treatment.
With slow-moving myeloma, stabilization can last for many years.
· Allogeneic ­ The infusion of bone marrow or stem cells from one individual (donor) to
stage: The extent of a cancer in the body.
another (recipient). A patient receives bone marrow or stem cells from a compatible,
though not genetically identical, donor.
staging: Doing exams and tests to learn the extent of the cancer in the body.
· Autologous ­ A procedure in which stem cells are removed from a patient's blood and then
are given back to the patient following intensive treatment.
stem cells: The immature cells from which all blood cells develop. Normal stem cells give
rise to normal blood components, including red cells, white cells, and platelets. Stem cells are
· Matched unrelated donor transplants (MUDs) ­ Refers to stem cell transplantation pro-
normally located in the bone marrow and can be harvested for transplant.
cedures in which the patient and the stem cells are genetically matched but are not from
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family members. This procedure is not recommended for myeloma patients because it
carries an unacceptably high mortality rate.
iMf PUBliCations
· Syngeneic ­ The infusion of bone marrow or stem cells from one identical twin into
another.
Patient Handbook
tumor: An abnormal mass of tissue that results from excessive cell division. Tumors perform
Concise Review of the Disease and Treatment Options
no useful body function. They may either be benign or malignant.
Understanding Series:
tumor marker: A substance in blood or other body fluids that may suggest that a person has
· Anemia and Fatigue
cancer.
· bal oon kyphoplasty and Myeloma-Induced vertebral Compression Fractures
vaccine: A preparation of killed microorganisms, living attenuated organisms, or living fully
· bisphosphonate Therapy
virulent organisms that is administered to produce or artificially increase immunity to a par-
ticular disease.
· Dexamethasone and other Steroids
· Revlimid® (lenalidomide)
virus: A small living particle that can infect cells and change how the cells function. Infection
with a virus can cause a person to develop symptoms. The disease and symptoms that are
· Serum Free Light Chain Assays
caused depend on the type of virus and the type of cells that are infected.
· Stem Cel Transplant
waldenström's macroglobulinemia: A rare type of indolent lymphoma that affects plasma
· Thalidomide
cells. Excessive amounts of IgM protein are produced. Not a type of myeloma.
· vELCADE® (bortezomib) for injection
white blood cells (wBC): General term for a variety of cells responsible for fighting invading
germs, infection, and allergy-causing agents. These cells begin their development in the bone
ASCO Highlights for Patients / Physicians
marrow and then travel to other parts of the body. Specific white blood cells include neutro-
ASH Highlights for Patients / Physicians
phils, granulocytes, lymphocytes, and monocytes.
Citings: Freelite/Hevylite
X-ray: High-energy electromagnetic radiation used in low doses to diagnose diseases and in
high doses to treat cancer.
Citings: Novel Therapies
Myeloma Today (IMF Quarterly Newsletter)
Myeloma Minute (IMF e-newsletter)
Publications of the International Myeloma Working Group
Publications of the IMF Nurse Leadership board
All are free of charge and available on the IMF website: www.myeloma.org,
or order from the IMF at 1-800-452-CURE (2873).
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Questions? Call the IMF Hotline:
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