"Until There is a Cure... There is the IMF."
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)
Dedicated to improving the quality of life of myeloma
patients while working towards prevention and a cure.
lomye yelom arrow
Prepared by Brian G.M. Durie, M.D.
© 2007 International Myeloma Foundation
taBle of Contents
WHAT IS MYELOMA?
BASIC FACTS ABOUT MYELOMA
WHY MYELOMA HAS TO BE TREATED
WHAT CAUSES THE MEDICAL PROBLEMS WITH MYELOMA? 6
DIFFERENT TYPES OF MYELOMA
STAGING OF MYELOMA
TESTING AT DIAGNOSIS
TREATMENT OF MYELOMA
INITIAL OR FRONTLINE THERAPY
IF FRONTLINE THERAPY IS NOT WORKING
QUESTIONS TO ASK YOUR DOCTOR
TERMS & DEFINITIONS
WHat Is MYeloMa?
Multiple myeloma is a bone marrow cancer. Despite periodic media attention, gen-
Myeloma is literally an "oma," or tumor, involving the "myelo," or blood-produc-
eral public awareness about myeloma is low. The intent of this booklet is to provide
ing cells in the bone marrow. The cel s that are af ected are plasma cel s (a type of
basic information and suggestions about how to cope with this disease.
white blood cell), which are our antibody- (immunoglobulin-) producing cells. A malig-
nant or cancerous plasma cel is cal ed a myeloma cel . Myeloma is cal ed "multiple"
The International Myeloma Foundation (IMF) is committed to providing educa-
since there are frequently multiple patches or areas in the bones 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 doc-
Myeloma affects the places where bone marrow is normally active in an adult.
tor. 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 dis-
are the extremities: that is the hands, feet, and lower arm/leg regions. This is very impor-
ease. Many patients go on to lead ful lives for years, even decades, after diagnosis. With
tant since the function of these critical areas is usual y ful y retained.
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
called MGUS. This is not a cancer, but a condition called 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.
TABLE 1: DEFINITIONS OF MGUS AND MYELOMA
BasIC faCts aBoUt MYeloMa
Although several things appear capable of causing or triggering myeloma, all of
the details are not known. Things associated with an increased risk of myeloma and
· Monoclonal protein present
related diseases are toxic chemicals (for example, agricultural chemicals and Agent
(i.e., no change in name)
· No underlying disease state.
Orange used in Vietnam), radiation (including atomic radiation), and several viruses
including human immunodeficiency virus (HIV), hepatitis viruses, human herpes virus
8 (HHV-8), and others. There is not a strong family tendency for myeloma; a few fami-
· Higher level of disease
lies do, however, have an increased predisposition to the disease.
than MGUS, but stil
no symptoms or organ
Myeloma occurs in adults. The average age of onset of myeloma is in one's early 60s.
Only 510% of patients are under the age of 40 years. It occurs more commonly in men
and in some racial groups, such as African-Americans.
· Monoclonal protein
There will be an estimated 19,000 new cases of myeloma in the U.S. in 2007. The
incidence ranges from ~0.51 /100,000 among Asians to as high as ~1012 /100,000
· One or more "CRAB"
among African-American men. At any one time there are over 50,000 myeloma patients
features of organ damage.*
undergoing treatment for their disease in the U.S.
*ORGAN DAMAGE CLASSIFIED AS "CRAB"
C calcium elevation (>10 mg/L)
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
WHat CaUses tHe MeDICal
Has to Be tReateD
PRoBleMs WItH MYeloMa
Myeloma, if left untreated, can cause bone damage, elevated blood calcium, low
Healthy plasma cel s produce immunoglobulins, which are complex proteins that we
blood counts (especially anemia), predisposition to infection, and kidney dam-
call "antibodies." Myeloma cells cannot make functioning antibodies, but instead pro-
age. Because the bones of the spine are often af ected and because myeloma proteins
duce a clonal protein, or immunoglobulin, that is known as a "monoclonal protein."
produced by myeloma cel s can damage nerves, it is common to have spine and nerve
All of the medical problems related to myeloma are caused by the build-up of
problems that may require urgent attention.
myeloma cells (see Table 2). Unlike other types of cancer, myeloma can present
patients with many strange complications because myeloma cells do not just pro-
In getting treatment for myeloma, it is important to distinguish between urgent
duce tumors, they release many proteins and other chemicals into the local micro-
problems such as bone damage, infection, kidney damage, or nerve pressure, which
environment of the bone marrow and directly into the blood stream.
need immediate attention, and overal planning to treat the disease. Sometimes urgent
care cannot and should not be delayed. However, early consultation with a hematolo-
· Local effects in the bone marrow. The effects in the bone marrow include a
gist/oncologist familiar with myeloma is encouraged. For example, options of emer-
reduction in blood cell production and damage to the surrounding bone. The
gency 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-
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
protein that is specific to the myeloma is released into the blood circulation.
there are any concerns, questions, or doubts, it is better to have these aired sooner
This specific immunoglobulin protein or monoclonal protein produced by
rather than later. Having a mutual y agreed-upon plan with your physician for ongoing
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.
TABLE 2: MEDICAL PROBLEMS RELATED TO MYELOMA
DIffeRent tYPes of
Effects Of Increased
Impact On Patient
Myeloma Cells In Bone
There are different types and subtypes of myeloma. These are based on the type of
Decrease in number and activity
immunoglobulin (protein) produced by the myeloma cell. Normally, the various
of red blood cel -producing
immunoglobulins have different functions in the body. Each immunoglobulin protein
is made up of two heavy chains and two light chains. (See Figure 1). There are five
High protein level
Abnormal or monoclonal
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
chains: k(K) and l(). The typing of myeloma (done with a test called "immunofixa-
myeloma cel s is released
· Possible kidney
tion") identifies both the heavy and light chains. Most myeloma patients, about 65%,
into the bloodstream and
have IgG (iG) type myeloma with k or l light chains. The next most common type is
can pass into the urine
(where it is cal ed Bence Jones
IgA (i A) type myeloma, also with either k or l light chains. (See Table 3). IgM, IgD, and
IgE myelomas are quite rare.
The myeloma cells activate osteo-
· Bone pain
· Thinning (osteoporosis) or
clast cel s, which destroy bone,
· Bone swel ing
FIGURE 1: IMMUNOGLOBULIN STRUCTURE
· Areas of more severe
and block osteoblast cells, which
· Fracture or col apse
damage cause lytic lesions,
normally repair damaged bone.
of a bone
fracture or collapse of a
High blood calcium
Release of calcium from damaged
· Mental confusion
bone into bloodstream
Approximately 30% of patients produce light chains in the urine (such as kappa light
chains) as wel as heavy and light chains (such as IgG kappa) in the blood. In about 10%
Reduced normal immune
The myeloma cells block produc-
· Susceptibility to
of patients, the myeloma cells produce only light chains and no heavy chains. This is
system function against
tion of normal antibodies against
cal ed "light chain" or "Bence Jones" myeloma. Rarely (in about 12% of patients) the
· Delayed recovery
myeloma cel s produce very little or no monoclonal protein of any type. This is cal ed
"non-secretory" myeloma. However, a recently developed test called the Freelite® test
(serum free light chain assay) can detect minute amounts of light chains in the blood
of most of these patients.
TABLE 3: T YPES OF MYELOMA
TABLE 4: THE DURIE AND SALMON STAGING SYSTEM
IgGK or IgG immunoglobulin G
(myeloma cells in billions/m2)*
with kappa or lambda light chains
lambda ( or L)
All of the following:
(low cell mass)
· Hemoglobin value >10g/dL
IgAK or IgA immunoglobulin A
· Serum calcium value normal
with kappa or lambda light chains
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
· Urine light chain M-component on
There are subtle differences in the behaviors of different types of myeloma. IgG myelo-
ma 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
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
*myeloma cells in whole body
are the most likely to cause kidney damage and/or lead to deposits of light chains in the
kidneys and/or on nerves or other organs. Depending upon the characteristics of the
One or more of the following:
light chain deposits, this condition is cal ed 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
· A: relatively normal renal function
(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)
TABLE 5: INTERNATIONAL STAGING SYSTEM (ISS)
Staging for Multiple Myeloma
testInG at DIaGnosIs
Table 7 summarizes the typical testing required at the time of diagnosis (baseline
TABLE 7: BASELINE TESTING
ß2M 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
to assess prognosis
or for a solitary plasmacytoma, direct biopsy of the tumor
(e.g., chromosomes, immune
mass is performed.
Note: ß2M = Serum ß2 microglobulin in mg/dL
typing, staining for amyloid
ALB = Serum albumin in g/dL
Chromosome analysis (cytogenetic testing) can reveal good
Several tests (assessments of so-cal ed prognostic factors, from the Greek words that
or poor chromosome features using direct and/or FISH
mean "knowing ahead") can be used to assess how aggressive the myeloma is in a given
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
· 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
(creatinine and BUN), calcium level, and LDH
· Serum ß2 microglobulin (S ß2M)
The higher the level the more advanced the stage.
3. Special protein testing
This shows the presence of the monoclonal myeloma
· Serum Albumin (S. Alb)
The lower the level the higher the stage.
· Serum protein
· The amount of the abnormal myeloma protein as wel as
the normal albumin protein level are measured.
· C-reative protein (CRP)
Increased with active disease
· Shows the type of myeloma protein [i.e., heavy chain
· Serum LDH (lactate dehydrogenase)
Increased with active disease
(G, A, D or E), light chain, kappa (K), lambda (L/)].
· Abnormal chromosomes on bone
Chromosome 13 deletions (13 q-) associated
· FREELITE® test
· Can be used to measure the amount of free kappa or
marrow cytogenetics and FISH
with shorter duration of remission as wel as
lambda if no SPEP or UPEP abnormality discovered.
(Fluorescent In Situ Hybridization)
other chromosomal abnormalities
TABLE 7: BASELINE TESTING CONT'D
tReatMent of MYeloMa
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
mended for active or symptomatic myeloma. The urgency of treatment depends upon
the exact problems faced by an individual patient.
To assess the presence, severity, and location of any areas
of bone damage.
TABLE 8: GOALS OF MYELOMA TREATMENT
X-rays are stil the gold standard in searching for myeloma
bone damage. A ful skeletal survey for myeloma using a series
of X-rays is needed to show loss or thinning of bone (osteo-
porosis or osteopenia caused by myeloma bone destruction),
lytic lesions, and/or any fracture or col apse of bone.
Countering the life-
· Plasmapheresis to thin the blood
and avoid stroke
Used when X-rays are negative and/or for more detailed
to body chemistry and
· Hemodialysis when kidney
testing of particular areas such as spine and/or brain. Can
the immune system
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.
· Radiation to stop bone
and increasing the
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
· Orthopedic surgery to repair
evaluation of smal areas of possible bone damage or nerve
and/or strengthen bone
· Therapy to kill malignant cells
Nuclear Medicine Scans
Routine bone scans used for other cancers. Not useful in
slowing or arresting the
throughout the body.
myeloma and should not be performed.
course of the disease
· Radiation to kill malignant cells
at a tumor site
A much more sensitive Whole Body scanning technique.
Useful for disease monitoring, especially for non-secretory
· Bone marrow transplants as a
means of delivering high-dose
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
the objective of many experimental treatments.
InItIal oR fRontlIne
TABLE 9: FRONTLINE TREATMENT OPTIONS
It is important for patients to set aside plenty of time to discuss the options with their
· An oral approach produc-
· Neuropathy and deep vein
ing remissions in 70% of
thrombosis (blood clots) are
hematologist or hematologist/oncologist. In addition to the baseline test results, one
· New gold standard for
· How much impact does the myeloma have on day-to-day functioning?
Pulse dexamethasone alone
Dexamethasone on an intensive
· Will the patient be able to work? Are plans in place to be off work as needed to
provides a substantial per-
schedule can be poorly tolerated
centage of the benefit of the
· How old is the patient? Are there any other medical problems?
· Taken by mouth
· Can cause bone marrow stem cel
· Can the patient tolerate chemotherapy?
· Well tolerated
damage and therefore reduce chanc-
· Produces excel ent
es of successful stem cel transplant
· Is high-dose chemotherapy with stem cell transplant an option?
remissions in about
· Full benefit occurs slowly over
60% of patients
· How well and how rapidly you respond to treatment varies from patient to
· Physicians very familiar
· Not ideal if prompt response
required and/or if stem cell
· All treatment decisions do not need to be made on "Day 1."
· It is generally best to keep the door open for stem cell transplantation if you
In combination with
The use of melphalan up front
melphalan, produces more
damages stem cells
feel it can be a future option for you.
rapid benefit than MP
· Although frontline clinical trials are available, you have to be completely com-
· Produces remission in
· Needs central line catheter for IV
fortable that you will be randomly assigned to one treatment versus another.
70% of patients
administration The catheter can
You may become "locked in" to future randomization and treatments. Make
· Doesn't damage normal
trigger infection and blood clot
sure you understand the full scope of the protocol.
· Can be basis for stem cell
· Vincristine can cause nerve damage
· If one treatment does not work, this does not mean that another treat-
ment cannot work extremely well and give an excellent remission.
Shows remarkable benefit
Produces neuropathy that is partially
with dexamethasone, Stem cel transplant stil
or completely reversible in this
and/or other agents, possible
TABLE 9: FRONTLINE TREATMENT OPTIONS TABLE CONT'D
A variety of other
· Combinations provide a
· More side effects than simpler
Treatments are available to al eviate the physical and emotional impact of the disease.
therapies are some-
Early use of supportive care measures is just as important as initiating frontline therapy.
times used such as
more aggressive approach,
if deemed necessary
· No added longer-term benefit
· Symptoms of active
· Side effects may both reduce
TABLE 10: SUPPORTIVE CARE
disease may be control ed
quality of life and compromise
more rapidly and quality
eligibility for new protocols
of first remission may be
· Blood transfusion (packed red
The treatments are simple, usual y
blood cel s: leukoreduced, virus
highly beneficial, and improve
screened) if anemia severe
feelings of well being.
· Erythropoietin if anemia mild to
(UK MRC protocol)
· Bisphosphonate (e.g., Aredia®
Relief of bone pain is important in
90mg IV over 2-4 hrs monthly;
itself and improves physical activity,
Zometa® 4 mg IV over 1545
which in turn promotes bone strength
and healing and improves emotional
· Pain medication as needed (e.g.,
well-being. Potential damage to kid-
Tylenol®, oral morphine deriva-
neys and jaws, though rare, can result
Further details about treatment options are available in other IMF publications.
tives, Fentanyl® "Pain Patch")
from chronic bisphosphonate therapy.
To order these, please contact the IMF or visit our website at www.myeloma.org.
Awareness is the key to prevention.
· Appropriate antibiotics
Although antibiotics should be
· Neupogen® if necessary to boost
selected and used with care, it is
low white blood cel count
extremely important that infections
· Intravenous gamma globulin for
be brought under control promptly.
Having an antibiotic on hand for
· Tests as needed to diagnose the
emergency use (especially if traveling)
exact type of infection (except
for dangerous biopsies/cultures)
should be performed.
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 major
such as planned walking or swimming, flexibility and strengthening exercises,
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.
· Herbal and vitamin supplements Talk to your doctor about using supple-
ments along with chemotherapy or other drug treatment. Drug interactions
Treatment decisions are critical y important to the survival and quality of life of the
can create medical problems.
myeloma patient. To make an informed decision, the patient needs to have the facts.
· Mental health Your mental health is critical as you move forward with
Some patients want to discuss al aspects of their situation, treatment, and prognosis.
planned treatment. Make sure you are comfortable with the treatment planned.
Others just want to know what to do next. Most doctors are sensitive to this and wil
Schedule an appointment with a mental health professional if you believe
vary their approach based on what they perceive to be the patient's wishes.
that you might be depressed, or if others are concerned that you might be
We encourage patients to be explicit about how deeply they want to get into the details
of the treatment decision. And, no matter how comfortable the patient feels with a doc-
· Regular sleep This is very important for your immune system.
tor, it is general y good practice to get more than one opinion before proceeding.
· Make adjustments As much as possible, reduce or eliminate stress in job,
family, or social situations. Management of the myeloma is the top priority
1. Get a complete description of the treatment program:
until remission and/or a stable situation has been achieved.
· What exactly is the treatment?
· What are the objectives of the treatment?
· Over what period will the treatment be given?
during treatment. Others may show up well after the treatment is completed.
· What is involved? How often must the patient visit a medical facility? Is hos-
· What side effects have been observed in patients receiving the treat-
pitalization required or a probability? What is the likely impact on the patient's
ment? When do they typically occur? In what percentage of patients do they
ability to function (i.e., work and play)? How do people feel before, during,
occur? How serious are the side effects? Are they life threatening? Are they
and after treatment? How do they look? What are typical recovery time frames?
painful? Are they permanent? How long do they last?
· What follow-up or maintenance programs are required?
· Are there treatments for the side effects? Do the treatments for the side
· What will the treatment program cost? Will it be covered by health insur-
effects have side effects?
4. There are always alternatives. You need to ask all of these questions for each
2. How well has this treatment worked for others in similar situations?
of the alternatives:
Effectiveness is measured in many different ways:
· What are the alternatives to the recommended treatment?
· How much experience is there with the treatment? How many patients
· What are the relative pros and cons of the alternatives?
have received it? How long have those patients been followed after the treat-
· What are the pros and cons of the alternative treatments vs. no treatment?
Because the disease is rare, there are a limited number of practitioners and centers spe-
· What are the odds of achieving a complete or partial remission? Which
cializing in myeloma. It is very common for a myeloma patient to seek a second opinion
factors suggest better or worse odds?
from a specialist at a research center while continuing to rely on a local referring physi-
· How long have the patients' remissions lasted? Which factors correlate to
cian to administer and monitor treatment.
long or to short remissions?
Making good decisions about treatment requires resourcefulness, careful questioning,
What would be the options in the event of a relapse? (These options may
change in the interim.)
serious thought, and courage. But, most of al , it requires that the patient and his/her
support group take charge of the process.
· What are reasonable expectations for relieving symptoms such as bone
pain, pathological fractures, anemia, fatigue, hypercalcemia? What are the
Because there is no known cure, because there are no guarantees, because every indi-
factors that predict how well these treatments will work for symptoms?
vidual is dif erent, the ultimate decision depends on the preferences and priorities of
· How long have people who have received the treatment survived? For
newer treatments, how many of the original group of patients are still alive?
3. Like most cancer treatments, myeloma treatments generally use strong drugs
and other measures aimed at destroying malignant cells and/or rebalancing
body chemistry. Typically, there are side effects. Some manifest themselves
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
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
asymptomatic myeloma: Myeloma that presents no signs or symptoms of disease. Also called
indolent, smoldering, or early myeloma.
anemia: A decrease in the normal number of red blood cells, usually below 10 g/dL with over
13-14 g/dL being normal. Myeloma in the bone marrow blocks red blood cell production,
axial skeleton: The skull, spine, and pelvis region of the skeleton.
causing shortness of 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.
Bence Jones: A myeloma protein present in urine. The amount of Bence Jones protein is
BUn (Blood Urea nitrogen): A measure of the urea level in the blood. Urea is cleared by
expressed in terms of grams per 24 hours. Normally a very small amount of protein (<0.1
the kidney. BUN is a laboratory blood test to assess how well the kidney is functioning.
g/24 h) can be present in the urine, but this is albumin rather than Bence Jones protein. The
Diseases, such as myeloma, which compromise kidney function, frequently lead to increased
presence of any Bence Jones protein is abnormal.
levels of 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
Beta 2 microglobulin (ß2M): A small protein found in the blood. High levels occur in
can invade nearby tissues and spread through the bloodstream and lymphatic system to
patients with active myeloma. Low or normal levels occur in patients with early myeloma
other parts of the body.
and/or inactive disease. Approximately 10% of patients have myeloma that does not pro-
duce ß2M. For these patients, ß2M testing cannot be used to monitor the disease. At the
Carcinogen: Any substance or agent that produces or stimulates cancer growth.
time of relapse, ß2M can increase before there is any change in the myeloma protein level.
Therefore, 90% of the time, ß2M is very useful for determining disease activity.
Cat or Ct [Computerized (axial) tomography scan]: A test using computerized X-rays
to create three-dimensional images of organs and structures inside the body, used to detect
Biopsy: The removal of a sample of tissue for microscopic examination to aid in diagnosis.
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
Catheter: A tube that is placed in a blood vessel to provide a pathway for drugs or nutrients.
(or destroyed) and protects against osteoclast activity.
A Central Venous Catheter is a special tubing that is surgically inserted into a large vein near
the heart and exits from the chest or abdomen. The catheter allows medications, fluids, or
Blood cells: Minute structures produced in the bone marrow; they include red blood cells,
blood products to be given and blood samples to be taken.
white blood cells, and platelets.
Cell: The basic unit of any living organism.
Blood count: The number of red blood cells, white blood cells, and platelets in a sample of
Cell differentiation: The process during which young, immature (unspecialized) cells take
on individual characteristics and reach their mature (specialized) form and function.
Bone marrow: The soft, spongy tissue in the center of bones that produces white blood
cells, 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
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
· Combination chemotherapy The use of more than one drug given in a chemotherapy
cells are checked to see whether they are cancerous. If cancerous plasma cells are found, the
regimen during cancer treatment.
pathologist estimates how much of the bone marrow is affected. Bone marrow biopsy is usu-
Chromosome: A strand of DNA and proteins in the nucleus of a cell. Chromosomes carry
ally done at the same time as bone marrow aspiration.
genes and function in the transmission of genetic information. Normally, human cells
contain 46 chromosomes.
Bone remodeling: The normal coordination (coupling) between osteoclast cells (which
resorb or destroy bone) and osteoblast cells (which create new bone matrix) to maintain a
Chronic: Persisting over a long period of time.
balanced state of bone production and destruction.
Clinical: Involving direct observation of a patient.
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
designed to find better ways to prevent, detect, diagnose, or treat cancer and to answer
are damaged, the serum level of creatinine builds up, resulting in an elevated serum creati-
nine. 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 semisolid 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
Dexamethasone: A powerful corticosteroid given alone or with other drugs.
usually the first human testing of a new treatment, although in phase I trials of combi-
nation therapies, the individual elements may already have been well tested. Patients in
Diagnosis: The process of identifying a disease by its signs and symptoms.
phase I 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 treat-
Dialysis: When a patient's kidneys are unable to filter blood, the blood is cleaned by passing
ment. All patients in a cohort get the same dose. The first cohort typically gets a very
it through a dialysis machine.
low dose, and 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
Disease-free survival: The length of time the patient survives without any detectable cancer.
is then taken to be the Maximum Tolerated Dose. This dose is then used in a phase II
Dlt (Dose limiting toxicity): Side-effects that are severe enough to prevent giving more
of the treatment.
· Phase II trial A trial designed to determine the response rate of a new therapy that
has already been tested in phase I trials. Typically, 14 to 50 patients with one type of
Dna: The substance of heredity; a large molecule that carries the genetic information that
cancer are treated to see how many have a response. Patients are usually required to
cells need to replicate and to produce proteins.
have advanced cancer that is refractory to any standard treatment, and in addition, they
must have measurable disease. If results from a phase II trial are promising enough, the
Drug resistance: The result of cells' ability to resist the effects of a specific drug.
treatment may then be tested in a phase III trial. If the results are obviously much better
than the standard treatment, then it may not be necessary to do a phase III trial, and
edema: Swelling; an abnormal accumulation of fluid in part of the body.
the treatment may become standard based on phase II trial results.
efficacy: The power to produce an effect; in cancer research `efficacy' refers to whether the
· Phase III trial A trial designed to compare two or more treatments for a given type
treatment is effective.
and stage of cancer. The end point of a phase III trial is usually survival or disease-free
survival. Phase III trials are usually randomized, so patients don't choose which treat-
electrophoresis: A laboratory test in which a patient's serum (blood) or urine molecules are
ment they receive. A typical phase III trial has 50 to thousands of patients. Some phase
subjected to separation according to their size and electrical charge. For myeloma patients,
III trials compare a new treatment that has had good results in phase II trials with an
electrophoresis of the blood or urine allows both the calculation of the amount of myeloma
older, well known, standard treatment. Other phase III trials compare treatments that
protein (M-protein) as well as the identification of the specific M-spike characteristic for
are already in common use. Some treatments in phase III trials may be available outside
each patient. Electrophoresis is used as a tool both for diagnosis and for monitoring.
the clinical trial setting.
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-
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 being used prophylactically before chemotherapy and as a
certain cells or organs.
supportive therapy after chemotherapy 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
Gene: A specific sequence of DNA or RNA; the biological unit of heredity located in a spe-
cause a number of symptoms, including loss of appetite, nausea, thirst, fatigue, muscle
cific place on a chromosome and found in all cells in the body. When genes are missing or
weakness, restlessness, and confusion. Common in myeloma patients and usually resulting
damaged, cancer may occur.
from bone 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, hyper-
Gene therapy: Treatment that alters genes. Using genes to stimulate the immune system.
calcemia is usually treated on an emergency basis using IV fluids combined with drugs to
In studies of gene therapy for cancer, researchers are trying to improve the body's natural
reduce bone destruction along with direct treatment for the myeloma.
ability 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 immunoglobu-
Genetic: Inherited; having to do with information that is passed from parents to children
lin, consists of two heavy chains, (for example of a G type) combined with two light chains,
through DNA in the genes.
which are either kappa or lambda. Therefore, the two most common subtypes of myeloma
have identical heavy chains (i.e. IgG kappa and IgG lambda). The terms heavy and light
Graft-versus-host disease (GVHD): A reaction of donated bone marrow against the recipi-
refer to the size or molecular weight of the protein, with the heavy chains being larger than
ent's own tissue.
the light chains. 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 mea-
IgM: Usually associated with Waldenstrom's macroglobulemia. In rare cases can be a type of
surement indicates anemia.
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
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.
Immunofixation: An immunologic test of the serum or urine used to identify proteins in
Interleukin: A naturally produced chemical released by the body or a substance used in
the blood. For myeloma patients, it enables the doctor to identify the M-protein type (IgG,
biological therapy. Interleukins stimulate the growth and activities of certain kinds of white
IgA, kappa, or lambda). The most sensitive routine immunostaining technique, it identifies
blood cells. Interleukin-2 (IL-2) is a type of biological response modifier that stimulates the
the exact heavy and light chain type of M-protein.
growth 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
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
destroying them. The classes of immunoglobulins are IgA, IgG, IgM, IgD, and IgE.
lDH: Lactate dehydrogenase, an enzyme that may be used to monitor myeloma activity.
Immunosuppression: Weakening of the immune system that causes a lowered ability to
lesion: An area of abnormal tissue change. A lump or abscess that may be caused by injury
fight infection and disease. Immunosuppression may be deliberate, such as in preparation
or disease, such as cancer. In myeloma, "lesion" can refer to a plasmacytoma or a hole in the
for 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
Immunotherapy: Treatment that stimulates the body's natural defenses to fight cancer. Also
blood cells (WBCs).
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.
Induction therapy: The initial treatment used in an effort to achieve remission in a newly
diagnosed myeloma patient.
lytic lesions: The damaged area of a bone that shows up as a dark spot on an X-ray when
enough of the healthy bone in any one area is eaten away. Lytic lesions look like holes in the
Informed consent: The process requiring a doctor to give a patient enough information
bone and are evidence that the bone is being weakened.
about a proposed procedure for the patient to make an informed decision about whether
or not to undergo it. The doctor must, in addition to explaining all procedures, address the
M proteins (M spike): Antibodies or parts of antibodies found in unusually large amounts
issues of risks, benefits, alternatives, and potential costs.
in the blood or urine of multiple myeloma patients. M spike refers to the sharp pattern that
occurs on protein electrophoresis when an M protein is present. Synonymous with mono-
Infusion: Delivering fluids or medications into the bloodstream over a period of time.
clonal protein and myeloma protein. (see "monoclonal" below)
Infusion pump: A device that delivers measured amounts of fluids or medications into the
Maintenance therapy: Drugs given to patients in remission to delay or prevent a relapse.
bloodstream over a period of time.
Malignant: Cancerous; capable of invading nearby tissue and spreading to other parts of the
Inhibit: To stop something, to hold in check.
Injection: Pushing a medication into the body with the use of a syringe and needle.
MDR (Multi Drug Resistance): A resistance to standard treatment, typically associated with
resistance to Adriamycin and vincristine, both chemotherapy drugs. The resistance is caused
Interferon: A naturally produced hormone (cytokine) released by the body in response to
by a buildup of the p-glycoprotein in the outer cell membrane of the myeloma cells. This
infection or disease which stimulates the growth of certain disease-fighting blood cells in the
results in drugs being kicked back out of the myeloma cell instead of building up and even-
immune system. Interferon can be artificially produced by genetic engineering techniques
tually killing that cell.
and used as a form of immunotherapy, primarily in the maintenance (plateau) phase to
block any regrowth of myeloma and thus delay or prevent relapse.
Melanoma: A cancer of the pigment-forming cells of the skin or the retina of the eye. Not
Myelosuppression: A decrease in the production of red blood cells, platelets, and some
associated with myeloma despite the similar-sounding name.
white blood cells by the bone marrow.
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.
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 environ-
protein is that it shows up as a sharp spike (M spike) in the serum electrophoresis test.
mental 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
oncologist: A doctor who specializes in treating cancer. Some oncologists specialize in a
they can 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
osteoclast: A cell found in the bone marrow at the junction between the bone marrow and
structures inside the body. Gives very fine resolution of soft tissues, especially encroach-
the bone that resorbs or breaks down old bone. In myeloma, the osteoclasts are over-stimu-
ments on the 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
osteoid: The protein product which becomes mineralized with calcium to form hard
Myelodysplastic syndrome: A condition in which the bone marrow does not function nor-
mally and does not produce enough blood cells. This condition may progress and become
osteonecrosis of the jaw: A previously rare jaw problem now being observed in a small
percentage of patients taking bisphosphonates. The condition produces pain, swelling, and
bone damage around the tooth sockets in the jaws. There is bone necrosis or loss of bone
Myeloid: Referring to myelocytes, a type of white blood cell. Also called myelogenous.
which can lead to loose teeth, sharp edges of exposed bone, bone spurs, and the breaking
Multiple myeloma is a non-myeloid cancer.
loose of small 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, numb-
ness or a "heavy jaw" feeling, or loosening of a tooth.
osteoporosis: Reduction in bone density typically associated with old age. Diffuse involve-
Port - Implanted: A catheter connected to a quarter-sized disc that is surgically placed just
ment of bones with myeloma produces what looks like osteoporosis on X-ray and bone
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 be drawn through a needle that is stuck into the disc.
Palliative treatment: Aimed to improve the quality of life by relieving pain and symptoms
of disease but not intended to alter its course.
Prognosis: The projected outcome or course of a disease; the chance of recovery; the life
Pathological fracture: A break in a bone usually caused by cancer or some disease condi-
tion. Occurs in myeloma-weakened bones, which can't bear normal weight or stress.
Progression-free survival: The time period during which the patient survives and the cancer
does not become worse. The improved survival of a patient that can be directly attributed
Pathology: The study of disease by the examination of tissues and body fluids under the
to the treatment given for the myeloma. This term identifies myeloma patients who are in
microscope. A doctor who specializes in pathology is called a pathologist.
complete remission versus those who have had an episode of relapse or progression.
Pet (Positron emission tomography) scan: A diagnostic test that uses a sophisticated
Progressive disease: Disease that is becoming worse, as documented by tests.
camera and computer to produce images of the body. PET scans show the difference
between healthy and abnormally functioning tissues.
Protocol: A detailed plan of treatment including the dose and schedule of any drugs used.
Placebo: An inert (inactive) substance often used in clinical trials for comparison with an
Precancerous: A term used to describe a condition that may, or is likely to become, cancer.
Radiation therapy: Treatment with x-rays, gamma rays, or electrons to damage or kill
Plasma: The liquid part of the blood in which red blood cells, white blood cells, and plate-
malignant cells. The radiation may come from outside the body (external radiation) or from
lets are suspended.
radioactive materials placed directly in the tumor (implant radiation).
Plasma cells: Special white blood cells that produce antibodies. The malignant cell in
Radiologist: A doctor who specializes in creating and interpreting images of areas inside the
myeloma. Normal plasma cells produce antibodies to fight infection. In myeloma, malig-
body. The images are produced with x-rays, sound waves, magnetic fields, or other types of
nant plasma cells produce large amounts of abnormal antibodies that lack the capability to
fight infection. 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, kid-
Recurrence: The reappearance of a disease after a period of remission.
ney damage, and nerve damage).
Red blood cells (erythrocytes): Cells in the blood that contain hemoglobin and deliver oxy-
Plasmacytoma: A collection of plasma cells found in a single location rather than diffusely
gen to and take carbon dioxide from all parts of the body. Red cell production is stimulated
throughout the bone marrow, soft tissue, or bone.
by a hormone (erythropoietin) produced by the kidneys. Myeloma patients with damaged
kidneys don't produce enough erythropoietin and can become anemic. Injections with syn-
Plasmapheresis: The process of removing certain proteins from the blood. Plasmapheresis
thetic erythropoietin can be helpful. Blood transfusion is another alternative, especially in
can be used to remove excess antibodies from the blood of multiple myeloma patients.
an emergency. Synthetic erythropoietin is being used prophylactically before chemotherapy
and as supportive therapy after chemotherapy to avoid anemia.
Platelet: One of the three major blood elements, others being the red blood cells and white
blood cells. Platelets plug up breaks in the blood vessel walls and release substances that
Refractory: Disease that is unresponsive to standard treatments.
stimulate blood clot formation. Platelets are the major defense against bleeding. Also called
Regression: The shrinkage of cancer growth.
Relapse: The reappearance of signs and symptoms of a disease after a period of improvement.
steroid: A type of hormone. Steroids are often given to patients along with one or more
anticancer drugs and appear to help to control the effects of the disease on the body.
Remission or response: Complete or partial disappearance of the signs and symptoms of
cancer. Remission and response are used interchangeably.
supportive care: Treatment given to prevent, control, or relieve complications and side
· Complete Remission (CR) CR is the absence of myeloma protein from the serum
effects and to improve the patient's comfort and quality of life.
and/or urine by standard testing; absence of myeloma cells from the bone marrow and/
or other areas of myeloma involvement; clinical remission and improvement of other
systemic therapy: Treatment using substances that travel through the bloodstream, reaching
laboratory parameters to normal. CR is not the same thing as a cure.
and affecting cancer cells all over the body.
· Partial Remission (PR) PR is a level of response less than CR. In SWOG studies, it
thrombocytes: See "Platelets."
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
250,000. If the platelet count is less than 50,000, bleeding problems could occur. Major
of cellular chemical activities. RNA is one of the two nucleic acids found in all cells the
bleeding is usually associated with a reduction to less than 10,000.
other 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.
transfusion: The transfer of blood or blood products.
shingles: See "Herpes zoster."
transplantation: Stem cells are used to rescue a patient's blood-forming potential following
side effects: Problems that occur due to drugs used for disease treatment. Common side
high-dose chemotherapy and/or radiation treatment. Transplant is not a treatment, but a
effects of cancer chemotherapy are fatigue, nausea, vomiting, decreased blood cell counts,
method of support to make high-dose treatment possible.
hair loss, and mouth sores.
· Bone marrow transplantation This term refers to the process of collecting stem cells
skeletal survey (metastatic survey): A series of plain X-rays of the skull, spine, ribs, pelvis,
from the bone marrow and infusing them into a patient. This term is used less fre-
and long bones to look for lytic lesions and/or osteoporosis.
quently today in myeloma as stem cells are now collected from the peripheral or circu-
stable disease: This describes patients who have some response to treatment but <50%
reduction in myeloma protein levels. Stable disease is not necessarily bad or sub-optimal
· Peripheral blood stem cell transplantation Doctors remove healthy stem cells from a
(as compared with CR or PR) provided the myeloma has stabilized and is not progressing.
patient's circulating blood system (not from the bone marrow) and store them before
With slow-moving myeloma, stabilization can last for many years.
the patient receives high-dose chemotherapy to destroy the cancer cells. The stem cells
are then returned to the patient, where they can produce new blood cells to replace cells
stage: The extent of a cancer in the body.
destroyed by the treatment.
· 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.
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
· Autologous A procedure in which stem cells are removed from a patient's blood and
are normally located in the bone marrow and can be harvested for transplant.
then are given back to the patient following intensive treatment.
· Matched unrelated donor transplants (MUDs) Refers to stem cell transplantation
procedures 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
tumor: An abnormal mass of tissue that results from excessive cell division. Tumors per-
form 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
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
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.
Waldenstrom's macroglobulemia: 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 invad-
ing 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
neutrophils, granulocytes, lymphocytes, and monocytes.
X-ray: High-energy electromagnetic radiation used in low doses to diagnose diseases and in
high doses to treat cancer.