"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)
ion
Tel: 818.487.7455
Fax: 818.487.7454
Email: TheIMF@myeloma.org
a
Website: www.myeloma.org
Foundata
Dedicated to improving the quality of life of myeloma
patients while working toward prevention and a cure.
lomye yelom arrow
M MoneB
ionalM
ofthe
ultiple
Prepared by Brian G.M. Durie, M.D.
© 2008 International Myeloma Foundation
Internat
M ancerC
2008/2009 Edition

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taBle of Contents
INTRODUCTION
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

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IntRoDUCtIon
WHat Is MYeloMa?
Multiple myeloma is a bone marrow cancer. Despite periodic media attention,
Myeloma is literally an "oma," or tumor, involving the "myelo," or blood-
general public awareness about myeloma is low. The intent of this booklet is to provide
producing cells in the bone marrow. The cel s that are af ected are plasma cel s
basic information and suggestions about how to cope with this disease.
(a type of white 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
The International Myeloma Foundation (IMF) is committed to providing educa-
"multiple" since there are frequently multiple patches or areas in bone where tumors or
tion and support for patients and families. This handbook provides a basic under-
lesions have developed. A single lesion is cal ed a solitary plasmacytoma.
standing of myeloma suf icient to al ow patients to make informed decisions about
treatment choices. The handbook is supplemental to the information given by the
Myeloma affects the places where bone marrow is normally active in an adult.
doctor. Caregivers, family, and friends may also find the information useful.
This marrow is in the hol ow area within the bones of the spine, skul , and pelvic bones,
the rib cage, and the areas around the shoulders and hips. The areas usual y not af ected
Although there is currently no cure for myeloma, it is an eminently treatable
are the extremities: that is the hands, feet, and lower arm/leg regions. This is very impor-
disease. Many patients go on to lead ful lives for years, even decades, after diagnosis.
tant since the function of these critical areas is usual y ful y retained.
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
Undetermined Significance. In MGUS, the myeloma cel s constitute fewer than 10% of
sometimes be more aggressive. While the doctor assesses each particular situation and
the bone marrow cel s. The risk of transition from MGUS to active myeloma is very low:
recommends the best approach, the patient plays a central role in helping make these
only a 1% chance each year of fol ow-up. Even if the myeloma cel s are at a higher level
individual treatment decisions. It is important that patients and their families be wel
of 10­30% of the total bone marrow, the growth rate can be very slow and represent
informed, ask questions, and give serious thought to alternative strategies or options.
indolent/smoldering or asymptomatic myeloma. Both MGUS and indolent myeloma
can change very slowly over a period of years and do not require active treatment. It
is very important to establish the correct diagnosis distinguishing MGUS and indolent
myeloma from active or symptomatic myeloma, which does require treatment.
1
2

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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
MGUS
MGUS
· Monoclonal protein
the details are not known. Things associated with an increased risk of myeloma and
(Monoclonal Gammopathy
(i.e., no change in name)
present.
related diseases are toxic chemicals (for example, agricultural chemicals and Agent
of Undetermined
· No underlying disease
Orange used in Vietnam as wel as a whole range of petrochemical compounds),
Significance)
state.
radiation (including atomic radiation), and several viruses including human immuno-
SMOLDERING or
ASYMPTOMATIC
· Higher level of disease
deficiency virus (HIV), hepatitis viruses, human herpes virus 8 (HHV-8), and others.
INDOLENT MYELOMA
MYELOMA
than MGUS, but stil
There is not a strong family tendency for myeloma; however, a few families do have an
no symptoms or organ
increased predisposition to the disease.
damage.*
Myeloma occurs in adults. The average age of onset of myeloma is in one's early 60s.
· Monoclonal protein
Only 5­10% of patients are under the age of 40 years. Myeloma occurs more commonly
MYELOMA
SYMPTOMATIC
present, and
in men and in some racial groups, such as African-Americans.
MYELOMA
· One or more "CRAB"
features of organ damage.*
There are approximately 20,000 new cases of myeloma in the U.S. each year. The
incidence ranges from ~0.5­1 /100,000 among Asians to as high as ~10­12 /100,000
*ORGAN DAMAGE CLASSIFIED AS "CRAB"
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)
B ­ bone disease (lytic lesions or osteoporosis)
ONE OR MORE required for diagnosis of SYMPTOMATIC MYELOMA
3
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WHY MYeloMa
WHat CaUses tHe MeDICal
Has to Be tReateD
PRoBleMs WItH MYeloMa
Myeloma, if left untreated, can cause bone damage, elevated blood calcium,
Healthy plasma cel s produce immunoglobulins, which are complex proteins that we
low blood counts (especially anemia), predisposition to infection, and kidney
call "antibodies." Myeloma cells do not make functioning antibodies, but instead pro-
damage. Because the bones of the spine are often affected and because myeloma pro-
duce a clonal protein, or immunoglobulin, that is known as a "monoclonal protein."
teins produced by myeloma cel s can damage nerves, it is common to have spine and
All of the medical problems related to myeloma are caused by the build-up
nerve problems that may require urgent attention.
of myeloma cells (see Table 2). Unlike other types of cancer, myeloma can present
patients with many strange complications because myeloma cel s do not just produce
In getting treatment for myeloma, it is important to distinguish between urgent
tumors, they release many proteins and other chemicals into the local microenviron-
problems such as bone damage, infection, kidney damage, or nerve pressure, which
ment of the bone marrow and directly into the blood stream.
need immediate attention, versus overal planning to treat the disease. Sometimes
urgent care cannot and should not be delayed. However, early consultation with a
· Local effects in the bone marrow. The effects in the bone marrow include a
hematologist/oncologist familiar with myeloma is encouraged. For example, options of
reduction in blood cell production and damage to the surrounding bone. The
emergency surgery versus radiation therapy can be discussed. Also, making sure that al
net results are the many common features of myeloma, such as anemia, predis-
treatment options are kept open for the future is an important consideration.
position to infection, bone pain, bone fractures, and elevated blood calcium.
Once urgent matters have been dealt with, overal plans can be discussed in more
· Effects outside the bone marrow. The effects outside the bone marrow are
detail. Frequently there is time to seek a second opinion or consultation with an expert
mostly due to the monoclonal protein produced by the myeloma cells. As the
to be assured that al options are careful y reviewed. Even if plans seem to be clear, if
myeloma cells build up in the bone marrow, the immunoglobulin or antibody
there are any concerns, questions, or doubts, it is better to have these aired sooner
protein that is specific to the myeloma is released into the blood circulation.
rather than later. Having a mutual y agreed-upon plan with your physician for ongoing
This specific immunoglobulin protein or monoclonal protein produced by
myeloma cells can cause tissue damage at distant sites; for example, kidney
treatment is tremendously important.
damage is not uncommon. The protein can interfere with blood clotting and/or
circulation, and can potentially 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

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TABLE 2: MEDICAL PROBLEMS RELATED TO MYELOMA
DIffeRent tYPes of
Effects Of Increased
Cause
Impact On Patient
MYeloMa
Myeloma Cells In Bone
Marrow
There are different types and subtypes of myeloma. These are based on the type of
Anemia (A*)
Decrease in number and activity
· Fatigue
immunoglobulin (protein) produced by the myeloma cell. Normally, the various
of red blood cel -producing
· Weakness
immunoglobulins have different functions in the body. Each immunoglobulin protein
cells.
is made up of two heavy chains and two light chains. (See Figure 1). There are five
High protein level
Abnormal or monoclonal
· Sluggish
types of heavy protein chains: G, A, D, E, and M. There are two types of light protein
(in blood and/or urine)
protein produced by the
circulation
chains: kappa () and lambda ( or L). The typing of myeloma (done with a test cal ed
myeloma cel s is released
· Possible renal or
"immunofixation" [IFE]) identifies both the heavy and light chains. Most myeloma
into the bloodstream and
kidney damage
patients, about 65%, have IgG (iG) type myeloma with or light chains. The next
can pass into the urine
(R*)
(where it is cal ed Bence Jones
most common type is IgA (iA) type myeloma, also with either or light chains.
protein).
(See Table 3). IgM, IgD, and IgE myelomas are quite rare.
Bone Damage (B*):
The myeloma cells activate osteo-
· Thinning (osteoporosis) or
clast cel s, which destroy bone,
· Bone pain
FIGURE 1: IMMUNOGLOBULIN STRUCTURE
· Areas of more severe
and block osteoblast cells, which
· Bone swel ing
Light chain
damage cause lytic lesions,
normally repair damaged bone.
· Fracture or col apse
Heavy chain
fracture or collapse of a
of a bone
vertebra
High blood calcium (C*)
Release of calcium from damaged
· Mental confusion
bone into bloodstream
· Dehydration
· Constipation
· Fatigue
Approximately 30% of patients produce light chains in the urine (such as kappa light
· Weakness
· Renal or kidney
chains) as wel as heavy and light chains (such as IgG kappa) in the blood. In about 10%
damage (R*)
of patients, the myeloma cel s produce only light chains and no heavy chains. This is
cal ed "light chain" or "Bence Jones" myeloma. Rarely (in about 1­2% of patients) the
Reduced normal immune
The myeloma cells block produc-
· Susceptibility to
system function against
tion of normal antibodies against
infection
myeloma cel s produce very little or no monoclonal protein of any type. This is cal ed
infection
infection
· Delayed recovery
"non-secretory" myeloma. However, the FreeliteTM test (serum free light chain assay)
from infection
can detect minute amounts of light chains in the blood of most of these patients.
*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
lambda ( or L)
STAGE I
All of the following:
600 billion*
(low cell mass)
· Hemoglobin value >10g/dL
IgA:
kappa ()
IgA or IgA immunoglobulin A
· Serum calcium value normal
(immunoglobulin A)
or
with kappa or lambda light chains
or <10.5mg/dL
lambda ( or L)
· Bone x-ray, normal bone structure (scale
0), or solitary bone plasmacytoma only
*Rarer types are IgD, IgE and IgM
· Low M-component production rates
**There are only 2 light chain types
IgG value <5g/dL; IgA value <3g/dL
There are subtle differences in the behaviors of different types of myeloma. IgG myelo-
· Urine light chain M-component on
ma has the usual features of myeloma. The IgA type can sometimes be characterized by
electrophoresis <4g/24h
tumors outside of the bone. The IgD type can be accompanied by plasma-cel leukemia
STAGE II
Fitting neither Stage I nor Stage III
600 to 1,200 billion*
and more frequently causes kidney damage. The light chain or Bence Jones myelomas
(intermediate
*myeloma cells in whole body
are the most likely to cause kidney damage and/or lead to deposits of light chains in the
cell mass)
kidneys and/or on nerves or other organs. Depending upon the characteristics of the
STAGE III
One or more of the following:
>1,200 billion*
light chain deposits, this condition is called either amyloid or light chain deposition.
(high cell mass) · Hemoglobin value <8.5g/dL
· Serum calcium value >12mg/dL
· Advanced lytic bone lesions (scale 3)
staGInG of MYeloMa
· High M-component production rates
IgG value >7g/dL IgA value >5g/dL
· Bence Jones protein >12g/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 previously most commonly used
SUBCLASS-
· A: relatively normal renal function
IFICATION
(serum creatinine value) <2.0 mg/dL
staging system is shown in Table 4, and shows the correlation between the extent of
(either A or B)
· B: abnormal renal function
the myeloma and the damage caused, such as bone disease or anemia. The outlook is
(serum creatinine value) >2.0 mg/dL
better when treatment is started early and bone disease or other complications can be
Examples: Stage IA (low cell mass with
prevented. The most current staging system is shown in Table 5, and is the result of the
normal renal function) Stage IIIB (high cell
collaboration of more than twenty research institutions world-wide.
mass with abnormal renal function)
9
10

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TABLE 5: INTERNATIONAL STAGING SYSTEM (ISS)
Staging for Multiple Myeloma
testInG at DIaGnosIs
STAGE
VALUES
Table 7 summarizes the typical testing required at the time of diagnosis (baseline
STAGE 1
2M <3.5
ALB 3.5
testing).
TABLE 7: BASELINE TESTING
STAGE 2
2M <3.5
ALB <3.5
TEST
PURPOSE
or
2M 3.5 ­ 5.5
Bone marrow biopsy
This is the single most critical test to determine the percent-
Special testing is done
age of myeloma cel s in the bone marrow. In Stage I disease
STAGE 3
2M >5.5
to assess prognosis
or for a solitary plasmacytoma, direct biopsy of the tumor
(e.g., chromosomes, immune
mass is performed.
Note: 2M = Serum 2 microglobulin in mg/L
typing, staining for amyloid)
ALB = Serum albumin in g/dL
Chromosome analysis (cytogenetic testing) can reveal good
or poor chromosome features using direct and/or FISH
Several tests (assessments of so-cal ed prognostic factors, from the Greek words that
analysis.
mean "knowing ahead") can be used to assess how aggressive the myeloma is in a given
Blood Testing
patient. In general, higher or abnormal test results indicate more active myeloma, and
possibly, less likelihood of having a long response with treatment (Table 6).
1. Complete blood count
· To assess presence/severity of anemia (low hemoglobin)
(CBC)
· To assess for low white cell count
TABLE 6: PROGNOSTIC FACTORS
· To assess for low blood platelet count
2. Chemistry panel
· Particularly important to assess kidney function
TEST
SIGNIFICANCE
(creatinine and BUN), albumin, calcium level, and LDH
· Serum 2 microglobulin (S 2M)
The higher the level the more advanced the stage
3. Special protein testing
This shows the presence of the monoclonal myeloma
protein.
· Serum Albumin (S Alb)
The lower the level the higher the stage
· Serum protein
· The amount of the abnormal myeloma protein.
· C-reative protein (CRP)
Increased with active disease
electrophoresis (SPEP)
· Serum LDH (lactate dehydrogenase)
Increased with active disease
· Immunofixation
· Shows the type of myeloma protein [i.e., heavy chain
(G, A, D or E), light chain, kappa (), lambda ()].
· Abnormal chromosomes on bone
Several chromosome deletions or translocations;
· FREELITE® test
· Can be used to measure the amount of free kappa or
marrow cytogenetics and FISH
can be associated with shorter duration of
lambda if no SPEP or UPEP abnormality discovered.
(Fluorescent In Situ Hybridization)
remission
11
12

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TABLE 7: BASELINE TESTING CONT'D
TEST
PURPOSE
tReatMent of MYeloMa
Urine Testing
Special protein testing similar
Shows the presence, amount, and type of abnormal
Deciding about treatment is the most important initial decision. As already emphasized,
to serum above:
myeloma protein in urine.
baseline testing, staging, and prognostic classification are essential. Treatment is recom-
· Urine Protein Electophoresis
(UPEP)
mended for active or symptomatic myeloma. The urgency of treatment depends upon
· Immunofixation
the exact problems faced by an individual patient.
Bone Testing
To assess the presence, severity, and location of any areas
of bone damage.
TABLE 8: GOALS OF MYELOMA TREATMENT
X-Rays
X-rays are stil the gold standard in searching for myeloma
TYPE OF
OBJECTIVE
EXAMPLES
TIME TO
bone damage. A ful skeletal survey for myeloma using a series
TREATMENT
DECIDE
of X-rays is needed to show loss or thinning of bone (osteo-
porosis or osteopenia caused by myeloma bone destruction),
Stabilizing
Countering the life-
· Plasmapheresis to thin the blood
lytic lesions, and/or any fracture or col apse of bone.
Hours to
threatening disruptions
and avoid stroke
Days
to body chemistry and
· Hemodialysis when kidney
MRI
Used when X-rays are negative and/or for more detailed
the immune system
testing of particular areas such as spine and/or brain. Can
function is impaired
reveal the presence and distribution of disease in the bone
· Drugs to reduce hypercalcemia
marrow when X-rays show no bone damage. Can also
(may include chemotherapy)
reveal disease outside of bone, which may be pressing on
nerves and/or spinal cord.
Palliative
Relieving discomfort
· Radiation to stop bone
Days to
and increasing the
destruction
Months
CT Scan
Used when X-rays are negative and/or for more detailed
patient's ability to
· Erythropoietin to relieve anemia
testing of particular areas. Especially useful for detailed
function
· Orthopedic surgery to repair
evaluation of smal areas of possible bone damage or nerve
and/or strengthen bone
pressure.
Remission-
Improving symptoms,
· Therapy to kill malignant cells
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 kill malignant cells
at a tumor site
FDG/PET Scan
A much more sensitive Whole Body scanning technique.
Useful for disease monitoring, especially for non-secretory
Curative
Permanent remission*
· Bone marrow transplants as a
Weeks to
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
* Although never achieved and confirmed, permanent remission remains
bisphosphonate therapy.
the objective of many experimental treatments.
13
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InItIal oR fRontlIne
TABLE 9A: FRONTLINE TREATMENT OPTIONS ­ Transplant Eligible
tHeRaPY
FRONTLINE
ADVANTAGES
DISADVANTAGES
THERAPY
VAD
· Produces remission in 70%
· Needs central line catheter for IV
It is important for patients to set aside plenty of time to discuss the options with their
(Vincristine/
of patients
administration. The catheter can trigger
Adriamycin/
· Doesn't damage normal
infection and blood clot complications
hematologist or hematologist/oncologist. In addition to the baseline test results, one
Dexamethasone)
stem cells
· Vincristine can cause nerve damage
must consider:
· Can be basis for stem cell
· New options are available that are
transplant
more effective and less toxic
· How much impact does the myeloma have on day-to-day functioning?
Dexamethasone plus · An oral approach producing · Neuropathy and deep vein thrombosis
Thalidomide*
remission in 70% of patients
(blood clots) are potential concerns
· Will the patient be able to work? Are plans in place to be off work as needed to
· New gold standard
undertake treatment?
for frontline induction
· How old is the patient? Are there any other medical problems?
Dexamethasone*
· Pulse dexamethasone alone
· Dexamethasone on an intensive
alone
provides a substantial
schedule can be poorly tolerated
· Can the patient tolerate potential treatments?
percentage of the benefit
of the full VAD
· Is high-dose chemotherapy with stem cell transplant an option?
R or RD or Rd
· Excellent response rates
· Revlimid® alone can result in
· How well and how rapidly one responds to treatment varies from patient to
(RevloDex)*
· Oral
less effective response
· Risk of blood clot problems; requires
patient.
(Revlimid® alone, with · Generally well-tolerated
Dexamethasone, or
and increasingly popular
aspirin or another blood thinner
· Possible reduced stem cell harvest
· All treatment decisions do not need to be made on "Day 1."
Revlimid® with low-
dose Dexamethasone)
· It is generally best to keep the door open for stem cell transplantation if you
VELCADE®**
· Shows remarkable benefit
· Produces neuropathy that is partial y
feel it can be a future option for you.
· Many combinations available
or completely reversible in this setting
· Preferred in cases of renal
· Although frontline clinical trials are available, you have to be completely com-
compromise/abnormal
fortable that you will be randomly assigned to one treatment versus another.
genetic features
You may become "locked in" to future randomization and treatments. Make
VTD
· Very high response rate
· Intravenous combination
sure you understand the full scope of the protocol.
(VELCADE®/
in recent phase III trial
· Potential for side effects: peripheral
Thalidomide/
· Excellent outcomes post-
neuropathy
· If one treatment does not work, this does not mean that another treat-
Dexamethasone)
transplant
ment cannot work extremely well and give an excellent remission.
More Complex
Excellent response rates
· Intravenous combinations
VELCADE® Combos
· Possible increased toxicities
(with Revlimid®,
with uncertain benefits
Doxil®, or other agents)
* Can be used with or without plan for harvest and transplant
15
** In June 2008, VELCADE®'s approval was expanded to include previously untreated
16
myeloma patients.

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TABLE 9B: FRONTLINE TREATMENT OPTIONS TABLE ­ Transplant Ineligible
FRONTLINE
ADVANTAGES
DISADVANTAGES
sUPPoRtIVe CaRe
THERAPY
MP
· Taken by mouth
· Can cause bone marrow stem cell
(Melphalan/
· Well tolerated
damage and therefore reduce chances
Treatments are available to al eviate the physical and emotional impact of the disease.
Prednisone)
· Produces excel ent remissions
of successful stem cell transplant
Early use of supportive care measures is just as important as initiating frontline therapy.
in about 60% of patients
· Full benefit occurs slowly over
· Physicians very familiar
several months
with protocol
· Not ideal if prompt response required
TABLE 10: SUPPORTIVE CARE
and/or if stem cell transplant planned
Dexamethasone
· In combination with
· The use of melphalan up-front
SYMPTOM
TREATMENT
COMMENTS
plus Melphalan
melphalan, produces more
damages stem cells
rapid benefit than MP
· Dexamethasone can be difficult
Fatigue and
· Blood transfusion (packed red
The treatments are simple, usual y
for older patients
weakness due
blood cel s: leukoreduced, virus
highly beneficial, and improve
to anemia
screened) if anemia severe
feelings of well being.
MPT
· Taken by mouth
· Same as for MP
· Erythropoietin if anemia mild to
(MP + thalidomide)
· Well tolerated
· Thalidomide has risks of neuropathy
· Higher remission rate than MP and/or blood clot problems (DVT)
moderate
VMP
· Generally well tolerated
· Same as for MP
· Bisphosphonate (e.g., Aredia®
Relief of bone pain is important in
(VELCADE® + MP)
· No blood clot risk
· VELCADE® is I.V.
Bone Pain
· Higher remission rate than MP · Significant risk of neuropathy
90mg IV over 2-4 hrs monthly;
itself and improves physical activity,
Zometa® 4 mg IV over 15­45
which in turn promotes bone strength
MPR
· Taken by mouth
· Risk of blood clot problems with
minutes monthly)
and healing and improves emotional
(MP + Revlimid®)
· Well tolerated
Revlimid®. Aspirin or another blood
· Pain medication as needed
well-being. Potential damage to kid-
· Higher remission rate than MP
thinner required.
(e.g., Tylenol®, oral morphine
neys and jaws, though rare, can result
derivatives, Fentanyl® "Pain
from chronic bisphosphonate therapy.
A variety of other
· Combinations provide a more · More side effects than simpler
therapies are sometimes
aggressive approach, if deemed
regimens
Patch")
Awareness is the key to prevention.
used such as Cytoxan®
necessary
· No added longer-term benefit
(cyclophosphamide) and · Symptoms of active disease
· Side effects may both reduce
· Appropriate antibiotics
Although antibiotics should be
Etoposide® (VP-16).
may be control ed more rapidly quality of life and compromise
Fever
Potential combinations
and quality of first remission
eligibility for new protocols
and/or
· Neupogen® if necessary to boost
selected and used with care, it is
include:
may be better
evidence
low white blood cel count
extremely important that infections
· VBMCP (M2 protocol)
of infection
· Intravenous gamma globulin for
be brought under control promptly.
· VMCP/VBAP
severe infections
Having an antibiotic on hand for
(SWOG protocol)
· Tests as needed to diagnose the
emergency use (especially if traveling)
· ABCM
exact type of infection (except
is recommended.
(UK MRC protocol)
for dangerous biopsies/cultures)
* In June 2008, VELCADE®'s approval was expanded to include previously untreated myeloma patients.
should be performed.
Further details about treatment options are available in other IMF publications.
17
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
physical activity is feasible or if adjustments need to be made because of bone
There are numerous treatment options beyond the scope of this introductory
disease and particular areas of bone damage. Usually, some physical activity
handbook. Emerging new therapies are increasingly available and can provide
such as planned walking or swimming, flexibility and strengthening exercises,
major benefit.
and/or a personalized yoga program, can be set up.
Please visit the IMF website at www.myeloma.org for more information and regular
· Diet ­ No specific diet has been developed for myeloma patients. This is an
updates or call the IMF at 800-452-CURE (2873).
area of ongoing research. In general, "healthy diet" recommendations from
other disease settings such as cardiac disease and cancer in general (e.g., breast
cancer) can be utilized. Caution should be used in two areas:
· Vitamin C ­ High doses (i.e., >1000 mg/day) may be counter-productive in
QUestIons to asK
myeloma and increase the risk of kidney damage.
YoUR DoCtoR
· Herbal and vitamin supplements ­ Talk to your doctor or oncology cen-
ter pharmacist about using supplements along with chemotherapy or other
Treatment decisions are critical y important to the survival and quality of life of the
drug treatment. Drug interactions can create medical problems. Most phar-
myeloma patient. To make an informed decision, the patient needs to have the facts.
macies have systems which identify potential interactions with supplements
Some patients want to discuss al aspects of their situation, treatment, and prognosis.
in addition to medications.
Others just want to know what to do next. Most doctors are sensitive to this and wil
· Mental health ­ Your mental health is critical as you move forward with
vary their approach based on what they perceive to be the patient's wishes.
planned treatment. Make sure you are comfortable with the treatment planned.
We encourage patients to be explicit about how deeply they want to get into the details
Schedule an appointment with a mental health professional if you believe
of the treatment decision. And, no matter how comfortable the patient feels with a
that you might be depressed, or if others are concerned that you might be
doctor, it is general y good practice to get more than one opinion before proceeding.
depressed.
· Regular sleep ­ This is very important for your immune system.
1. Get a complete description of the treatment program:
· Make adjustments ­ As much as possible, reduce or eliminate stress in job,
· What exactly is the treatment?
family, or social situations. Management of the myeloma is the top priority
· What are the objectives of the treatment?
until remission and/or a stable situation has been achieved.
· Over what period will the treatment be given?
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· What is involved? How often must the patient visit a medical facility?
3. Like most cancer treatments, myeloma treatments generally use strong drugs
Is hospitalization required or a probability? What is the likely impact on
and other measures aimed at destroying malignant cells and/or rebalancing body
the patient's ability to function (i.e., work and play)? How do people feel
chemistry. Typically, there are side effects. Some manifest themselves during
before, during, and after treatment? How do they look? What are typical
treatment. Others may show up well after the treatment is completed.
recovery time frames?
· What side effects have been observed in patients receiving the treatment?
· What follow-up or maintenance programs are required?
When do they typically occur? In what percentage of patients do they occur?
· What will the treatment program cost? Will it be covered by health
How serious are the side effects? Are they life threatening? Are they painful? Are
insurance?
they permanent? How long do they last?
2. How well has this treatment worked for others in similar situations?
· Are there treatments for the side effects? Do the treatments for the side
Effectiveness is measured in many different ways:
effects have side effects?
· How much experience is there with the treatment? How many patients
4. There are always alternatives. You need to ask all of these questions for each
have received it? How long have those patients been followed after the treat-
of the alternatives:
ment?
· What are the alternatives to the recommended treatment?
· What are the odds of achieving a complete or partial remission? Which
· What are the relative pros and cons of the alternatives?
factors suggest better or worse odds?
· What are the pros and cons of the alternative treatments vs. no treatment?
· How long have the patients' remissions lasted? Which factors correlate
Because the disease is rare, there are a limited number of practitioners and centers spe-
to long or to short remissions?
cializing in myeloma. It is very common for a myeloma patient to seek a second opinion
· What would be the options in the event of a relapse? (These options
from a specialist at a research center while continuing to rely on a local referring physi-
may change in the interim.)
cian to administer and monitor treatment.
· What are reasonable expectations for relieving symptoms such as bone
Making good decisions about treatment requires resourcefulness, careful questioning,
pain, pathological fractures, anemia, fatigue, hypercalcemia? What are
the factors that predict how well these treatments will work for symptoms?
serious thought, and courage. But, most of al , it requires that the patient and his/her
support group take charge of the process.
· How long have people who have received the treatment survived? For
newer treatments, how many of the original group of patients are still alive?
Because there is no known cure, because there are no guarantees, because every indi-
vidual is dif erent, the ultimate decision depends on the preferences and priorities of
the patient.
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angiogenesis inhibitors: Compounds that attempt to cut off the blood supply to tumors.
teRMs & DefInItIons
antibiotics: Drugs used to treat infection.
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
accrual: The process of enrolling patients in a clinical research study (trial), or the number of
can bind only to a specific antigen. The purpose of this binding is to help destroy the antigen.
patients already enrolled in a trial or anticipated to enroll in a trial.
Antibodies can work in several ways, depending on the nature of the antigen. Some antibod-
ies disable antigens directly. Others make the antigen more vulnerable to destruction by other
acute: A sudden onset of symptoms or disease.
white blood cells.
albumin: Simple water-soluble proteins that are found in blood serum and many other ani-
anti-emetic agent: A drug that prevents or controls nausea and vomiting.
mal and plant tissues.
antifungal agent: A drug used to treat fungal infections.
alkylating agent: A chemotherapeutic agent such as melphalan or cyclophosphamide.
Alkylating refers to the way in which these agents cross-link the DNA of myeloma cells and
antigen: Any foreign substance (such as a bacteria, virus, toxin or tumor) that, when intro-
block cell division.
duced into or arising in the body, causes the immune system to produce natural antibodies.
allogeneic: See "Transplantation."
antineoplastic agent: A drug that prevents, kills, or blocks the growth and spread of cancer
cells.
amyloidosis: A condition in which myeloma light chains (Bence Jones proteins) are deposited
in tissues and organs throughout the body. This occurs more commonly with lambda versus
appendicular skeleton: The long bones (i.e., arms and legs), which are attached to spine,
kappa Bence Jones proteins. In patients with amyloidosis, the light chain proteins bind to
chest and pelvis.
certain tissues such as heart, nerves and kidney rather than being excreted out of the body
through the kidneys.
apoptosis: A normal cellular process involving a genetically programmed series of events lead-
ing to the death of a cell.
analgesic: Any drug that relieves pain. Aspirin and acetaminophen are mild analgesics.
aspiration: The process of removing fluid or tissue, or both, from a specific area.
analog: A chemical compound that is structurally similar to another but differs slightly in
composition.
asymptomatic myeloma: Myeloma that presents no signs or symptoms of disease. Also called
indolent, smoldering, or early myeloma.
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
axial skeleton: The skull, spine, and pelvis region of the skeleton.
breath, weakness, and tiredness.
B cells: White blood cells that develop into plasma cells in the bone marrow and are the source
anesthesia: Loss of feeling or awareness. Local anesthesia causes loss of feeling in a part of the
of antibodies. Also known as B lymphocytes.
body. General anesthesia puts the person to sleep.
Basophil: A type of white blood cell. Basophils are granulocytes.
angiogenesis: Blood vessel formation, which usually accompanies the growth of malignant
tissue, including myeloma.
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Bence Jones: A myeloma monoclonal protein present in urine. The amount of Bence Jones
BUn (Blood Urea nitrogen): A measure of the urea level in the blood. Urea is cleared by the
protein is expressed in terms of grams per 24 hours. Normally a very small amount of protein
kidney. BUN is a laboratory blood test to assess how well the kidney is functioning. Diseases,
(<0.1 g/24 h) can be present in the urine, but this is albumin rather than Bence Jones protein.
such as myeloma, which compromise kidney function, frequently lead to increased levels of
The presence of any Bence Jones protein is abnormal.
BUN.
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 virual 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.
Clinical: Involving direct observation of a patient.
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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
neoplasia: Abnormal new growth of cells.
and form secondary tumors, the cells in the metastatic tumor are like those in the original
(primary) tumor. This term is commonly used to describe a disease process in solid tumors
neoplasm: A new growth of tissue or cells; a tumor that can be referred to as benign or
(e.g., breast, prostate) and not in myeloma, which is a blood-related cancer.
malignant.
MGUs (Monoclonal Gammopathy of Undetermined significance): A benign condition in
neutropenia: A reduced level of neutrophils. Cytotoxic chemotherapy has a tendency to
which the M protein is present but there is no underlying disease.
induce neutropenia. In contrast, lymphocytes which are more important in viral infections,
tend not to be affected by cytotoxic treatment. Neutropenia can be prevented or reduced
Molecule: The smallest particle of a substance that retains all the properties of the substance
using a synthetic hormone called G-CSF (e.g. Neupogen).
and is composed of one or more atoms.
neutrophil: A type of white blood cell necessary to combat bacterial infection.
Monoclonal: A clone or duplicate of a single cell. Myeloma develops from a single malignant
plasma cell (monoclone). The type of myeloma protein produced is also monoclonal; a single
oncogene: A gene or DNA sequence that normally directs cell growth, but which can also
form rather than many forms (polyclonal). The important practical aspect of a monoclonal
promote or allow the uncontrolled growth of cancer if damaged (mutated) by an environmen-
protein is that it shows up as a sharp spike (M spike) in the serum electrophoresis test.
tal exposure to carcinogens, or if damaged or missing because of an inherited defect. A gene
that has the potential to cause a normal cell to become cancerous.
Monoclonal antibodies: Artificially manufactured antibodies specifically designed to find and
bind to cancer cells for diagnostic or treatment purposes. They can be used alone, or they can
oncologist: A doctor who specializes in treating cancer. Some oncologists specialize in a
be used to deliver drugs, toxins, or radioactive material directly to tumor cells.
particular type of cancer treatment.
Monocyte: A type of white blood cell.
osteoblast: The cell that produces osteoid, which becomes mineralized with calcium to form
new hard bone.
MRI (Magnetic Resonance Imaging): A diagnostic test that uses magnetic energy, rather than
X-ray energy, to produce detailed two- or three-dimensional images of organs and structures
osteoclast: A cell found in the bone marrow at the junction between the bone marrow and
inside the body. Gives very fine resolution of soft tissues, especially encroachments on the
the bone that resorbs or breaks down old bone. In myeloma, the osteoclasts are over-stimu-
spinal cord, but is less accurate for bone lesions.
lated while osteoblast activity is blocked. The combination of accelerated bone resorption and
blocked new bone formation results in lytic lesions.
MtD (Maximum tolerated Dose): The highest dose of a treatment that most people can
safely withstand.
osteoid: The protein product which becomes mineralized with calcium to form hard bones.
Myelodysplastic syndrome: A condition in which the bone marrow does not function nor-
osteonecrosis of the jaw: A previously rare jaw problem now being observed in a small per-
mally and does not produce enough blood cells. This condition may progress and become
centage of patients taking bisphosphonates. The condition produces pain, swelling, and bone
acute leukemia.
damage around the tooth sockets in the jaws. There is bone necrosis or loss of bone which can
lead to loose teeth, sharp edges of exposed bone, bone spurs, and the breaking loose of small
Myeloid: Referring to myelocytes, a type of white blood cell. Also called myelogenous.
bone spicules or dead bone. A case definition is 3 months with non-healing exposed bone.
Multiple myeloma is a non-myeloid cancer.
Symptoms may not be obvious at first, or may include pain, swelling, numbness or a "heavy
jaw" feeling, or loosening of a tooth.
Myelosuppression: A decrease in the production of red blood cells, platelets, and some white
blood cells by the bone marrow.
osteoporosis: Reduction in bone density typically associated with old age. Diffuse involve-
ment of bones with myeloma produces what looks like osteoporosis on X-ray and bone
density measurement.
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Palliative treatment: Aimed to improve the quality of life by relieving pain and symptoms of
Port ­ Implanted: A catheter connected to a quarter-sized disc that is surgically placed just
disease but not intended to alter its course.
below the skin in the chest or abdomen. The catheter is inserted into a large vein or artery
directly into the bloodstream. Fluids, drugs, or blood products can be infused, and blood can
Pathological fracture: A break in a bone usually caused by cancer or some disease condition.
be drawn through a needle that is stuck into the disc.
Occurs in myeloma-weakened bones, which can't bear normal weight or stress.
Prognosis: The projected outcome or course of a disease; the chance of recovery; the life
Pathology: The study of disease by the examination of tissues and body fluids under the
expectancy.
microscope. A doctor who specializes in pathology is called a pathologist.
Progression-free survival: The time period during which the patient survives and the cancer
Pet (Positron emission tomography) scan: A diagnostic test that uses a sophisticated cam-
does not become worse. The improved survival of a patient that can be directly attributed to
era and computer to produce images of the body. PET scans show the difference between
the treatment given for the myeloma. This term identifies myeloma patients who are in com-
healthy and abnormally functioning tissues.
plete remission versus those who have had an episode of relapse or progression.
Placebo: An inert (inactive) substance often used in clinical trials for comparison with an
Progressive disease: Disease that is becoming worse, as documented by tests.
experimental drug.
Protocol: A detailed plan of treatment including the dose and schedule of any drugs used.
Plasma: The liquid part of the blood in which red blood cells, white blood cells, and platelets
are suspended.
Precancerous: A term used to describe a condition that may, or is likely to become, cancer.
Plasma cells: Special white blood cells that produce antibodies. The malignant cell in myelo-
Radiation therapy: Treatment with x-rays, gamma rays, or electrons to damage or kill malig-
ma. Normal plasma cells produce antibodies to fight infection. In myeloma, malignant plasma
nant cells. The radiation may come from outside the body (external radiation) or from radio-
cells produce large amounts of abnormal antibodies that lack the capability to fight infection.
active materials placed directly in the tumor (implant radiation).
The abnormal antibodies are the monoclonal protein, or M protein. Plasma cells also produce
other chemicals that can cause organ and tissue damage (i.e., anemia, kidney damage, and
Radiologist: A doctor who specializes in creating and interpreting images of areas inside the
nerve damage).
body. The images are produced with x-rays, sound waves, magnetic fields, or other types
of energy.
Plasmacytoma: A collection of plasma cells found in a single location rather than diffusely
throughout the bone marrow, soft tissue, or bone.
Recurrence: The reappearance of a disease after a period of remission.
Plasmapheresis: The process of removing certain proteins from the blood. Plasmapheresis can
Red blood cells (erythrocytes): Cells in the blood that contain hemoglobin and deliver oxy-
be used to remove high levels of monoclonal myeloma protein from the blood of multiple
gen to and take carbon dioxide from all parts of the body. Red cell production is stimulated
myeloma patients.
by a hormone (erythropoietin) produced by the kidneys. Myeloma patients with damaged
kidneys don't produce enough erythropoietin and can become anemic. Injections with
Platelet: One of the three major blood elements, others being the red blood cells and white
synthetic erythropoietin can be helpful. Blood transfusion is another alternative, especially
blood cells. Platelets plug up breaks in the blood vessel walls and release substances that
in an emergency. Synthetic erythropoietin is a supportive therapy used during anti-myeloma
stimulate blood clot formation. Platelets are the major defense against bleeding. Also called
treatment to avoid anemia.
thrombocytes.
Refractory: Disease that is unresponsive to standard treatments.
Regression: The shrinkage of cancer growth.
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Relapse: The reappearance of signs and symptoms of a disease after a period of improvement.
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
Remission or response: Complete or partial disappearance of the signs and symptoms of
normally located in the bone marrow and can be harvested for transplant.
cancer. Remission and response are used interchangeably.
· Complete Remission (CR) ­ CR is the absence of myeloma protein from the serum and/or
steroid: A type of hormone. Steroids are often given to patients along with one or more anti-
urine by standard testing; absence of myeloma cells from the bone marrow and/ or other
cancer drugs and appear to help to control the effects of the disease on the body.
areas of myeloma involvement; clinical remission and improvement of other laboratory
parameters to normal. CR is not the same thing as a cure.
supportive care: Treatment given to prevent, control, or relieve complications and side effects
and to improve the patient's comfort and quality of life.
· Very Good Partial Remission (VGPR) ­ VGPR is just less than CR, that is, when myeloma
protein levels are reduced by 90%, but not gone.
systemic therapy: Treatment using substances that travel through the bloodstream, reaching
· Partial Remission (PR) ­ PR is a level of response less than CR. In SWOG studies, it has
and affecting cancer cells all over the body.
meant >50% and <75% response. In other studies it has meant >50% response.
thrombocytes: See "Platelets."
Rna (ribonucleic acid): Any of various nucleic acids that are associated with the control of
cellular chemical activities. RNA is one of the two nucleic acids found in all cells ­ the other
thrombocytopenia: A low number of platelets in the blood. The normal level is 150,000-
is DNA (deoxyribonucleic acid). RNA transfers genetic information from DNA to proteins
250,000. If the platelet count is less than 50,000, bleeding problems could occur. Major
produced by the cell.
bleeding is usually associated with a reduction to less than 10,000.
serum osteocalcin: A protein produced and secreted by osteoblasts when they are making
tnf (tumor necrosis factor): A type of biological response modifier that can improve the
osteoid. A low level reflects active myeloma. A higher than normal level reflects more stable
body's natural response to disease.
myeloma.
toxins: Poisons produced by certain animals, plants, or bacteria.
shingles: See "Herpes zoster."
transfusion: The transfer of blood or blood products.
side effects: Problems that occur due to drugs used for disease treatment. Common side
effects of cancer chemotherapy are fatigue, nausea, vomiting, decreased blood cell counts, hair
transplantation: There are several different types of transplantation.
loss, and mouth sores.
· Bone marrow transplantation ­ This term refers to the process of collecting stem cells
from the bone marrow and infusing them into a patient. This term is used less frequently
skeletal survey (metastatic survey): A series of plain X-rays of the skull, spine, ribs, pelvis, and
today in myeloma as stem cells are now collected from the peripheral or circulating
long bones to look for lytic lesions and/or osteoporosis.
blood.
stable disease: This describes patients who have some response to treatment but
· Peripheral blood stem cell transplantation ­ Doctors remove healthy stem cells from a
<50% reduction in myeloma protein levels. Stable disease is not necessarily bad or sub-optimal
patient's circulating blood system (not from the bone marrow) and store them before
(as compared with CR or PR) provided the myeloma has stabilized and is not progressing.
the patient receives high-dose chemotherapy to destroy the cancer cells. The stem cells
With slow-moving myeloma, stabilization can last for many years.
are then returned to the patient, where they can produce new blood cells to replace cells
destroyed by the treatment.
stage: The extent of a cancer in the body.
· Allogeneic ­ The infusion of bone marrow or stem cells from one individual (donor) to
staging: Doing exams and tests to learn the extent of the cancer in the body.
another (recipient). A patient receives bone marrow or stem cells from a compatible,
though not genetically identical, donor.
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· 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.
· Matched unrelated donor transplants (MUDs) ­ Refers to stem cell transplantation pro-
cedures in which the patient and the stem cells are genetically matched but are not from
family members. This procedure is not recommended for myeloma patients because it
carries an unacceptably high mortality rate.
· Syngeneic ­ The infusion of bone marrow or stem cells from one identical twin into
another.
tumor: An abnormal mass of tissue that results from excessive cell division. Tumors perform
no useful body function. They may either be benign or malignant.
tumor marker: A substance in blood or other body fluids that may suggest that a person has
cancer.
Vaccine: A preparation of killed microorganisms, living attenuated organisms, or living fully
virulent organisms that is administered to produce or artificially increase immunity to a par-
ticular disease.
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
caused depend on the type of virus and the type of cells that are infected.
Waldenström's macroglobulinemia: A rare type of indolent lymphoma that affects plasma
cells. Excessive amounts of IgM protein are produced. Not a type of myeloma.
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
marrow and then travel to other parts of the body. Specific white blood cells include neutro-
phils, granulocytes, lymphocytes, and monocytes.
X-ray: High-energy electromagnetic radiation used in low doses to diagnose diseases and in
high doses to treat cancer.
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