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CML Glossary & Abbreviations Index

We

know, it's just a bowl of alphabet soup out there. The letters all

start to blend together, and you can't remember the difference between

ABL and WBC. We hope this helps. Just click on the abbreviation, and

you'll be taken to its definition. Or just scan the entire listing. A * B * C

* D * E * F * G * H * I * J * K * L * M * N * O * P * Q * R * S * T * U * V * W

* X * Y * Z * ABL* ACOR* ALL * AML * ANC * ARA-C * BCR-ABL * BMA * BMB * BMT *

CBC * CCR * CML * CR * DLI * DX * FISH * FTI * GVHD * HX * IFN * MIU * MUD *

NSAID * PCR * PH+(-) * RBC * STI * SCT * WBC A ABL.

A gene (named for a researcher whose last name was Abelson) on human

chromosome #9, involved in normal white blood cell replication. ABL

only causes trouble when it detaches and joins chromosome #22, creating

the BCR-ABL gene known to cause chronic myelogenous leukemia. Absolute

neutrophil count (ANC).

A measure of how many white blood cells you have to fight infections.

Your ANC is the total number of neutrophil cells per microliter of

blood. It is calculated by using the total white blood count (WBC) and

the percentage of neutrophils shown on the laboratory " differential. "

Normal ANC is around 2,500, but is still considered safe at 1,000 or

higher. See more in Understanding Blood. Accelerated phase.

The middle phase of CML, occurring between chronic and blast phase. The

actual definition of accelerated phase is somewhat variable. CML is

often considered accelerated when your blasts are between 10 percent

and 30 percent. Evidence of acceleration includes recurrence of certain

clinical findings such as enlarged spleen, night sweats, fatigue, etc.;

increased difficulty in controlling the blood cell counts; increases in

certain cell types such as basophils, eosinophils, and especially

blasts; and the appearance of new chromosomal abnormalities on

cytogenetic analysis. Any one of these may (or may not) be cause for

concern, but the appearance of two or more in combination makes the

diagnosis of accelerated phase more likely. Untreated, the accelerated

phase lasts from a couple of months to year or more before progressing

to blast phase. ACOR. The Association of Cancer Online Resources. Provides

information and listservs about cancer. Acute lymphocytic leukemia (ALL). A

type of leukemia in which too many infection-fighting white

blood cells called lymphocytes are found in the blood and bone marrow. Learn

more. Additive.

When patients take two or more drugs at the same time that produce the

same biological effect, the resulting effect generally follows one of

three patterns — additive effect, synergism, or potentiation. If drugs

have an additive effect it means that they combine to produce an

overall effect that is equal to the sum of their individual effects

(for example: 3 + 2 = 5). That is, the combined effect of using two

drugs together is no better than using the drugs separately. Allogeneic. Refers

to getting bone marrow (or other body tissue) from another person, not your own

body. Allo means other. Also see Autologous. Acute myelogenous leukemia (AML).

A type of leukemia in which cancer cells are found in the blood and

bone marrow. AML affects the blasts that are developing into white

blood cells called granulocytes. In AML, the blasts do not mature and

become too numerous. These immature blast cells are then found in the

blood and the bone marrow. AML is also called acute nonlymphocytic

leukemia or ANLL. Learn more. Angiogenesis.

The growth of new blood vessels, a process that occurs in the healthy

body for healing wounds and for restoring blood flow to tissues after

injury. In diseases like cancer, the body loses the ability to maintain

balanced angiogenesis. New blood vessels feed diseased tissues, destroy

normal tissues, and in the case of some cancers, the new vessels can

allow tumor cells to escape into the circulation and lodge in other

organs (tumor metastases). Angiogenesis inhibitor drugs are a new class

of drugs targeted against this abnormal process to block or slow tumor

growth. Visit the Angiogenesis Foundation to learn more. Apoptosis.

Programmed cell death. Normal cells are " programmed " by your body to

die after a certain amount of time or a certain number of cell

divisions. Cancerous cells lose this ability and go on living and

dividing indefinitely as long as they get the nutrition they need,

creating a huge build up of cells in your body, unless countered by

medications. ARA-C. Cytosine arabinoside, a chemotherapy drug often used to

treat CML and other leukemias. Learn more.

ASH. The American Society of Hematology.

Represents over 10,000 clinicians and scientists committed to further

the understanding, diagnosis, treatment, and prevention of disorders

affecting the blood, bone marrow, and the immunologic, hemostatic and

vascular systems, by promoting research, clinical care, education,

training, and advocacy in hematology. Publishes the journal Blood. AST.

Aspartate aminotransferase. A blood test used to detect liver inflammation.

Autologous. Refers to getting tissue (or marrow) back from yourself (auto means

self)

in a transplant. In an autologous stem cell transplant, for example,

your marrow cells are harvested and the " good " ones separated from the

" bad. " Your marrow is then destroyed with radiation and chemotherapy

and the autologous cells re-transfused. Avascular necrosis.

The death of the body's bone. It's a rare complication of some drug

therapies, including use of interferon, and is seen in patients whose

platelet count remains abnormally high despite treatment. B

BCR-ABL.

The abnormal gene that characterizes the leukemic stem cells of most

people with chronic myelogenous leukemia. For CML to occur the " abl "

gene (named after a researcher named Abelson) comes unglued from its

usual location on chromosome #9, and sticks itself to the " bcr "

(breakpoint cluster region) of chromosome #22, thus creating the bcr-abl hybrid.

About 95 percent of CML patients have this chromosome, as well as patients with

some other types of leukemia. See a patient's photo. Blast phase.

The third phase of CML after chronic and accelerated phases,

characterized by the presence of increasing numbers of highly immature

blood cells ( " blasts " or " myeloblasts " ) in the blood and marrow,

usually more than 30 percent. Blast phase is often fatal within months.

However, new treatments show promise in prolonging survival. Bone marrow

aspiration (BMA).

A procedure in which liquid contents of your bone marrow are withdrawn

(aspirated) through a needle. This procedure is used to make a

diagnosis and to follow progress of treatment. Bone marrow biopsy (BMB).

Similar to a bone marrow aspiration, but performed with a slightly

different piece of equipment. It is used when a chunk of marrow itself

is needed for diagnostic purposes, or when a BMA is unsuccessful

because the marrow is too fibrotic, that is, when there is too much

fibrous tissue and not enough to pull up through the aspiration needle.

Bone marrow

transplant (BMT).

A procedure in which your cancerous bone marrow is killed of with high

doses of chemotherapy and radiation and then replaced with healthy

donor marrow (sometimes called a stem cell transplant). C Chemotherapy.

The treatment or control of cancer using drugs that interfere with the

growth and multiplication of cancerous cells. Most chemo drugs are

targeted at a specific phase of the cell cycle and only kill cells that

are both multiplying and in that particular phase. Chimeric.

Generally refers to a combination or hybrid. In CML, the BCR-ABL gene

is a chimeric gene, or the combination of two other parts. Chromosome.

In a cell nucleus, a structure containing a molecule of DNA that

transmits genetic information. The normal number of chromosomes for

humans is 46. The chromosomal mutation leading to CML involves

chromosomes number 9 and 22. Chronic myelogenous leukemia (CML). A type of

leukemia in which too many white blood cells grow in the bone marrow. Learn

more. Clonal evolution.

The development of chromosomal mutations that occurs in untreated CML

or other leukemias, and leads to progression of the disease. Complete blood

count (CBC).

A blood test that measures white and red blood cells, platelets,

hemoglobin, and other cells. In CML a " white cell differential " is

usually performed along with the CBC. This tells which of several kinds

of white cells are present and in what proportion. See more in Understanding

Blood. Complete cytogenetic response (CCR). One of the types of positive

responses to treatment of leukemia. In

this case, it is the complete absence of leukemic (Ph+) cells in the bone

marrow of CML patients by either conventional or FISH cytogenetic testing..

Cytogenetics.

An evaluation of the genetic material of leukemia patients. Two types

of cytogenetics, " conventional " and FISH, are used to diagnose and

follow the course of CML. Conventional cytogenetics (so-called because

it's been around a long time) is a microscopic exam of up to 25 marrow

cells in a phase of cell division when your chromosomes can be clearly

seen and differentiated. Cytogenetic response (CR).

A response to treatment of CML that occurs in the marrow, rather than

just in the blood. There are 3 levels of cytogenetic response: 1) just

plain cytogenetic response (CR); 2) Major cytogenetic response (MCR);

and complete cytogenetic response (CCR). If the number of Ph+

chromosomes decreases at all during treatment, you have achieved a

cytogenetic response (CR); if your Ph+ percentage drops to 35 percent

or less, it is considered a major cytogenetic response (MCR); 0% Ph+ is

a complete cytogenetic response (CCR). D Donor leukocyte infusion (DLI).

A procedure to treat the relapse of a bone marrow transplant by taking

immune system cells from the original donor and transfusing them to the

leukemia patient. Learn more. Dx. The abbreviation for diagnosis; often found

in your medical files. E

Enzyme.

A protein that catalyzes changes in other biological substances. Too

many white cells are produced in CML because of an abnormal tyrosine

kinase enzyme whose sole activity is sticking phosphate molecules onto

tyrosine molecules. F Fluorescence in situ hybridization (FISH).

A powerful molecular cytogenetics technique that uses a

fluorescent-labeled DNA probe to determine the presence or absence of a

particular segment of DNA — the BCR-ABL gene in the case of CML. It

combines the ability to identify a specific gene or gene region

(molecular) with direct visualization of the cells and/or chromosomes

under the microscope (cytogenetics). In the FISH test, about 200 of

your cells are generally examined. The examined cells are usually from

your bone marrow but also can be from your peripheral blood (that is,

the blood in your veins). FISH can detect the BCR-ABL gene in CML

patients even when your bone marrow shows you are

Philadelphia-chromosome negative. Farnesyl transferase inhibitor (FTI).

Not to be confused with STI. FTI is an experimental drug that that

inhibits a cancer-causing gene known as ras, which causes cancer by

producing a growth-stimulating protein that signals uncontrolled cell

division. FTIs are being studied in CML and other cancers. G Gleevec.

A new drug used in the treatment of CML and other cancers that works by

inhibiting the enzyme that causes the cancer. Imatinib mesylate is a

protein-tyrosine kinase inhibitor that inhibits the Bcr-Abl tyrosine

kinase, the constitutive abnormal tyrosine kinase created by the

Philadelphia chromosome abnormality in CML. It inhibits proliferation

and induces apoptosis in Bcr-Abl positive cell lines as well as fresh

leukemic cells from Philadelphia chromosome positive chronic myeloid

leukemia. In colony formation assays using ex vivo peripheral blood and

bone marrow samples, imatinib shows inhibition of Bcr-Abl positive

colonies from CML patients. Learn more.

Also known as Glivec (outside of the United States) and STI571. Gleevec

(imatinib mesylate) is made by Novartis. Read more on the Novartis site. Graft

vs. host disease (GVHD).

A collection of ailments that sometimes arises after bone marrow

transplants, when the donor's immune system (the " graft " ) attacks

various tissues (the gut, skin, etc.) of the patient. H Hematologic response.

Normalization of the white blood cell counts in the blood, though not

necessarily in the bone marrow. The response can be partial (reduction

in white cells, but not down to normal range) or complete (white blood

count is at or 10 or 12 — the normal range varies by lab). Hematologist. A

physician who specializes in disorders of the blood (heme means blood in Greek),

including blood cancers such as leukemia. Heme-onc. Short for

hematologist-oncologist, a doctor who specializes in the treatment of both blood

diseases and solid cancers. Hydrea.

A chemotherapy drug — generic name is hydroxyurea — which is often used

first in the treatment of CML which works to kill leukemic white blood

cells and bring elevated white blood counts (WBCs) back toward normal. Learn

more. Hx. The abbreviation for history (as in your medical history). I

Interferon (IFN).

A glycoprotein chemical produced normally by mammalian cells in order

to fight infection and cancer. It is now produced by recombinant DNA

techniques and used as a drug to treat CML and other cancers. Also

known as Intron-A. Learn more. L

Leukemia. A cancer of the blood. Literally means " white blood. " Leukocyte.

Another name for white blood cell (leukos means white; cytos means cell in

Greek). M Million international units (MIU). A dosage amount, such as 5miu

(also written MU, or million units). Matched unrelated donor (MUD).

A bone marrow donor whose HLA matches the patients, but who is not

related to the patient. Most transplant patients do not find donors

from within their own families and must rely on strangers. Learn more.

Myelofibrosis.

Abnormal replacement of blood stem cells in the bone marrow with

fibrous tissue. Myelofibrosis occurs as a complication of CML and its

treatments, especially interferon. Multiple myeloma. A malignant disorder of

plasma. Multiple myeloma frequently is associated with bone pain and

susceptibility to infection. Learn more. N Nonmyeloablative.

A bone marrow transplant in which the patient's marrow is not

completely destroyed prior to receiving the donor's marrow or stem

cells (myelo means marrow; ablative means destructive). Nonsteroidal

anti-inflammatory drug (NSAID).

Drugs used to treat inflammation and fever. NSAID drugs mainly inhibit

the body's ability to synthesize prostaglandins. Prostaglandins are a

family of hormone-like chemicals, some of which are made in response to

cell injury. Learn more.

O Oncogene. A gene that has mutated, causing cells to become cancerous.

Oncologist. Cancer specialist (oncos means cancer in Greek). P Pegyllated

interferon.

A type of interferon (IFN) that has PolyEthylene Glycol (PEG) molecules

attached to it. This gives IFN a longer half-life in the body, and is

therefore hoped to reduce the drug's toxicity and increase its

effectiveness. Polymerase chain reaction (PCR). A very sensitive test used to

detect very

low levels of bcr-abl in marrow stem cells. Philadelphia chromosome (PH).

The hallmark of CML. This is the term used to describe the abnormal

appearing human chromosome 22, which is found in 95 percent of people

who have CML. It results from a mutation that involves the swapping of

genetic material between chromosomes 9 and 22 (see bcr-abl).

If you have this chromosome, you are considered Ph+, or Ph-positive. If

you don't, you are Ph-, or Ph-negative. The proportion of Ph+ to Ph-

cells is used to track progress in treating CML: If the number of Ph+

chromosomes decreases at all during treatment, you have achieved a

cytogenetic response (CR); if your Ph+ percentage drops to 35 percent

or less, it is considered a major cytogenetic response (MCR); 0% Ph+ is

a complete cytogenetic response (CCR). Platelets.

Fragments of a much larger cell, the megakaryocyte, that stays in the

bone marrow after it differentiates and matures from the stem cell. The

platelets leave the bone marrow and circulate throughout the body. When

stimulated by substances from damaged tissue, the platelets release

substances necessary to help blood clot.

R Red blood cells (RBC).

Highly specialized cells that have been " stripped " of everything,

including the nucleus, that might get in the way of doing their major

job, transporting oxygen Remission. Abatement or lessening in severity of the

symptoms, signs and laboratory abnormalities of a disease. S Signal

transduction inhibitor (STI).

One of the most exciting types of molecules in cancer research, STIs

mostly inhibit enzymes that carry out the actions that make cancer

cells behave as they do: multiplying too fast, living too long,

invading other tissues, etc. Gleevec is a type of STI. Stem cell. A

progenitor, or " primitive " cell ancestor, of almost all the blood cell types.

These immature cells

are found in the bone marrow and develop into red cells, white cells, or

platelets. Learn more. Stem cell transplant (SCT). See bone marrow transplant.

T Translocation.

A process in which a bit of genetic material from one chromosome swaps

places with a bit from another chromosome. In CML, a piece (called

" abl " ) from chromosome 9 is translocated onto a segment (called " bcr " )

on chromosome 22, creating the bcr-able oncogene. Tyrosine kinase. An enzyme

involved in communication within cells, or signaling pathways. It is an abnormal

tyrosine kinase that causes L. W White blood count (WBC).

One of three different types of cell. There are five distinctly

different kinds of white blood cells: neutrophils, monocytes,

lymphocytes, eosinophils, and basophils. Some have the ability to

change with needs and situations in the body. These cells can leave the

bloodstream, sliding out through the vessel walls and attacking

invaders at the site of an infection.

Thanks to Dr. Rockefeller for creating the bulk of this glossary.

a (Bobby) Doyle Brecksville, Ohio, USA DX 05/1995 02/2000 - Gleevec

Trial/OHSU 06/2002 - Gleevec/Trisenox

Trial/OHSU 06/2003 - Gleevec/Zarnestra Trial/OHSU 04/2004 - Sprycel

Trial/MDACC, CCR in 10

months 09/2006 -  out of CCRhasEML = false;

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