CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0393/03932.txt

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$Unique_ID{BRK03932}
$Pretitle{}
$Title{Leukemia, Chronic Lymphocytic}
$Subject{Leukemia Chronic Lymphocytic Chronic Lymphatic Leukemia CLL Hairy
Cell Leukemia Hodgkin's Disease Non-Hodgkin's Lymphoma Waldenstrom
Macroglobulinemia }
$Volume{}
$Log{}

Copyright (C) 1991, 1992 National Organization for Rare Disorders, Inc.

858:
Leukemia, Chronic Lymphocytic

** IMPORTANT **
It is possible the main title of the article (Chronic Lymphocytic
Leukemia) is not the name you expected.  Please check the SYNONYMS listing on
the next page to find alternate names and disorder subdivisions covered by
this article.

Synonyms

     Chronic Lymphatic Leukemia
     CLL

Information on the following diseases can be found in the Related
Disorders section of this report:

     Hairy Cell Leukemia
     Hodgkin's Disease
     Non-Hodgkin's Lymphoma
     Waldenstrom Macroglobulinemia

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the Resources
section of this report.

Chronic Lymphocytic Leukemia is a malignant blood disorder in which there
is an increased number of white blood cells formed in the lymphoid tissue.
This uncontrolled buildup and enlargement of lymphoid tissue can occur in
various sites of the body such as the lymph nodes, spleen, bone marrow, and
lungs.  There are many different forms of Leukemia which are all
characterized by an overabundance of white blood cells.  In Chronic
Lymphocytic Leukemia the disease occurs in the lymphoid tissue.

The lymph vessels, which return fluids to the circulatory system, and the
lymph nodes, which are a mass of tissue separated into compartments by
connective tissue, make up the immune system.  The lymph nodes serve as
filters, removing foreign particles, tissue debris, and bacterial cells from
the circulation.  When this system is not working properly, the body's
defenses cannot fight off foreign particles.

In the majority of cases, Chronic Lymphocytic Leukemia is the result of a
rapid production of B lymphocyte cells (a short-lived type of white blood
cell that is responsible for the production of vertebrate serum proteins that
include antibodies).  A small percentage of Chronic Lymphocytic Leukemia
cases stem from the overproduction of T lymphocyte cells (a type of white
blood cell that have a long life and are important in the resistance of
disease).

Symptoms

One quarter of the patients with Chronic Lymphocytic Leukemia have no
symptoms when first diagnosed.  The disease is discovered during a routine
exam or blood test.

Early signs of Chronic Lymphocytic Leukemia may be fatigue, weight loss,
loss of appetite (anorexia), labored breathing, low-grade fever, a feeling of
fullness in the abdomen due to an enlarged spleen, and night sweats.
Bacterial infections such as skin infections, fluid and inflammation of the
lungs (pneumonia), and inflammation of the sinuses (sinusitis) often occur.

As the disorder advances, the patient loses the ability to fight off
infections.  Viral infections become an increasing concern.  An abnormally
high sensitivity to insect bites may also occur.

In the later stages of the disorder, the liver, spleen, and lymph nodes
may steadily increase in size.  Chronic Lymphocytic Leukemia may also invade
other tissues such as the skin, eye socket (orbit), mucous membrane that
lines the inside of the eyelids (conjunctivae), lungs, sacs that line the
chest (pleura), heart, and gastrointestinal tract.  Swelling and a yellow
pigment of the skin (jaundice) may also occur.

Causes

Like most other forms of leukemia, the exact cause of Chronic Lymphocytic
Leukemia is not known.  When the disorder results from a rapid production of
B Lymphocyte cells, it is often inherited through autosomal dominant genes.
(Human traits including the classic genetic diseases, are the product of the
interaction of two genes for that condition, one received from the father and
one from the mother.  In dominant disorders, a single copy of the disease
gene (received from either the mother or father) will be expressed
"dominating" the normal gene and resulting in appearance of the disease.  The
risk of transmitting the disorder from affected parent to offspring is 50%
for each pregnancy regardless of the sex of the resulting child.)  Chronic
Lymphocytic Leukemia occurs in multiple family members more often than any
other type of leukemia.  Sibling, especially brothers, seem to have the
highest occurrence.

Other types of leukemia can occur from exposure to radiation.  However,
the cause is unknown in most cases.

Affected Population

Chronic Lymphocytic Leukemia is twice as common in males as in females.  This
is the most common type of leukemia occuring in multiple family members.  The
average age of patients is sixty, and the occurrence increases with age.
Chronic Lymphocytic Leukemia almost never affects children and is rare under
the age of thirty.

In the United States, three out of every 100,000 people will be afflicted
with this disorder.

Related Disorders

Symptoms of the following disorders can be similar to those of Chronic
Neutropenia.  Comparisons may be useful for a differential diagnosis.

Hairy Cell Leukemia is a type of blood cancer characterized by the presence
of abnormal mononuclear blood cells called "hairy cells," and by a deficiency
of other blood cell elements.  Symptoms of this disorder may include fatigue,
weight loss, abdominal pain, weakness, and easy bruising.  (For more
information on this disorder, choose "Hairy Cell Leukemia" as your search
term in the Rare Disease Database.

Hodgkin's Disease is a form of cancer of the lymphatic system, especially
the lymph nodes (places where lymphatic vessels unite).  Fever, night sweats,
swollen glands, and weight loss may occur.  Most often a lymph node in the
neck is affected.  (For more information on this disorder, choose "Hodgkin's
Disease" as your search term in the Rare Disease Database).

Infectious Mononucleosis is a very prevalent disorder characterized by
fever, fatigue, swollen glands, and an abnormally large number of lymphocytes
(type of white blood cells) in the blood.  It is caused by the Epstein-Barr
virus.  This disorder is most prevalent in places where young people live in
close quarters such as colleges or military.

Non-Hodgkin's Lymphomas are a group of cancers of the lymphatic system.
Swollen lymph nodes in the neck or groin occur and usually spread throughout
the body.  Symptoms of this disorder may be anemia (abnormally low levels of
red blood cells), weight loss, fever, night sweats, and weakness.  (For more
information on this disorder, choose "Non-Hodgkin's Lymphoma" as your search
term in the Rare Disease Database).

Waldenstrom's Macroglobulinemia is a malignant lymph node and blood cell
disorder.  Large quantities of homogenous immunoglobulin-M (IgM) protein
molecules are present in the blood.  The most frequent symptoms are an
enlarged spleen and liver, abnormalities of the peripheral lymph glands,
weakness, anemia, fatigue, and excessive bleeding, especially from the nose
and mouth.  This disorder tends to run in families and occurs mainly in older
males.  (For more information on this disorder, choose "Waldenstrom" as your
search term in the Rare Disease Database).

Therapies:  Standard

Treatment of Chronic Lymphocytic Leukemia includes platelet transfusions
which are used for bleeding associated with a persistent decrease in the
number blood platelets (thrombocytopenia).  When anemia is present,
transfusions of packed red blood cells are usually given.  Antibiotics are
used to combat bacterial infections usually related to a decrease in the
number of leukocytes (lymphopenia) and a low level of gammaglobulin in the
blood.

Radiation, anticancer, and corticosteroid drugs may be used to prevent
the spread of white cells in the lymph tissues.

Genetic counseling may be of benefit for patients and families if they
have the hereditary form of this disorder.

Therapies:  Investigational

Various antibodies (MoAbs) derived from a single cell are being used in large
quantities against the spreading of Chronic Lymphocytic Leukemia.  MoAbs
combined with other drugs are also being tested at this time.

The orphan drug Marinol is currently under investigation as a treatment
for Chronic Lymphocytic Leukemia.  The drug is manufactured by Warner Lambert
Company, 2800 Plymouth Rd., Ann Arbor, MI, 48105-2430.

The orphan product Technetium Tc-99m Murine Monoclonal Antibody (IgG2a)
to BCE (Immuraid-LL-2(99mTc) is being investigated for use in evaluating the
extent of disease in patients with Chronic Lymphocytic Leukemia and some
forms of non-Hodgkin's type Lymphoma.  The product is sponsored by
Immunomedics, Inc., 150 Mt. Bethel Rd., Warren, NJ, 07059.

This disease entry is based upon medical information available through
September 1992.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Chronic Lymphocytic Leukemia, please contact:

     National Organization for Rare Disorders
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     Leukemia Society of America
     733 Third Ave.
     New York, NY  10017
     [phone]

     American Cancer Society
     1599 Clifton Rd., NE
     Atlanta, GA  30329
     [phone]

     NIH/National Cancer Institute
     9000 Rockville Pike, Bldg. 31, Rm. 1A2A
     Bethesda, MD 20892
     1-800-4-CANCER

The National Cancer Institute has developed PDQ (Physician Data Query), a
computerized database designed to give the public, cancer patients and
families, and health professionals quick and easy access to many types of
information vital to patients with this and many other types of cancer.  To
gain access to this service, call:

     Cancer Information Service (CIS)
     1-800-4-CANCER
     In Washington, DC and suburbs in Maryland and Virginia, 636-5700
     In Alaska, 1-800-638-6070
     In Oahu, Hawaii, [phone] (Neighbor islands call collect)

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

MENDELIAN INHERITANCE IN MAN, 8th ed.:  Victor A. McKusick; Johns Hopkins
University Press, 1986.  Pp. 570-71.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W.B. Saunders Co., 1988.  Pp. 994-98.

HEMATOLOGY, 4th Ed.:  William J. Williams, et al., Editors; McGraw-Hill,
Inc., 1990.  Pp. 1005-20.