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1994-01-17
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$Unique_ID{BRK03783}
$Pretitle{}
$Title{Graft vs Host Disease}
$Subject{Graft vs Host Disease GVHD Acute GVHD Chronic GVHD Ulcerative Colitis
Lichen Planus}
$Volume{}
$Log{}
Copyright (C) 1991, 1992, 1993 National Organization for Rare Disorders,
Inc.
840:
Graft vs Host Disease
** IMPORTANT **
It is possible that the main title of the article (Graft vs Host Disease)
is not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
GVHD
Disorder Subdivisions:
Acute GVHD
Chronic GVHD
Information on the following diseases can be found in the Related
Disorders section of this report:
Ulcerative Colitis
Lichen Planus
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.
Graft vs Host Disease (GVHD) is a rare disorder that can strike persons
whose immune system is suppressed and have either received a blood
transfusion or a bone marrow transplant. Symptoms may include skin rash,
intestinal problems similar to colitis, and liver dysfunction.
Symptoms
The most frequent signs of Graft vs Host Disease (GVHD) occur after a blood
transfusion or bone marrow transplant. They are dermatitis (a skin rash),
gastrointestinal problems (diarrhea, nausea and abdominal pain) and poor
liver function. There may also be involvement of kidneys, lungs, eyes,
mouth, musculoskeletal system and heart. GVHD affects about 60% of all bone
marrow transplant and transfusion patients whose immune system was suppressed
before treatment. Immunosuppression can occur as a result of certain drugs,
radiation or certain diseases. Cancer patients and organ transplant
recipients often use drugs that suppress their immune systems.
Disorder Subdivisions:
Acute GVHD usually occurs in the first 100 days after blood transfusion
or bone marrow transplant. The first symptoms are usually mild skin rash,
liver dysfunction and intestinal problems, or they may show very severe skin
problems, diarrhea, nausea, abdominal pain and liver failure.
Chronic GVHD usually persists long after a transfusion or bone marrow
transplant. The signs and symptoms are similar to those of the Acute GVHD,
but in addition to the skin, abdomen and liver problems, Chronic GVHD may
also involve the eyes, mouth, lungs and musculoskeletal system.
Causes
GVHD is caused by the recipient's immune system's response to the lymphoid
cells in the donor's blood that react against the recipient's own blood
cells. GVHD can be acute (sudden) or chronic (long lasting). In cases of
bone marrow transplants the recipient usually undergoes radiation to destroy
their own diseased bone marrow, and their immune system is drastically
weakened when they receive the donor's bone marrow. Cancer patients
undergoing chemotherapy are also at high risk of getting GVHD from blood
transfusions.
Affected Population
GVHD affects males and females of all ages who have been immunosuppressed
before being given either blood transfusions or bone marrow transplants.
Related Disorders
Symptoms of the following disorders can be similar to those of Graft vs Host
Disease. Comparisons may be useful for a differential diagnosis:
Lichen Planus is a recurrent, itchy, inflammatory eruption of the skin
which is characterized by small separate, angular spots that may join
together into rough scaly patches. It is often accompanied by oral lesions.
The initial attack persists for weeks or months, and intermittent recurrences
may be noted for years. Moderate to severe itching may be present, and it
often does not respond to treatment. (For more information on this disorder,
choose "Lichen Planus" as your search term in the Rare Disease Database).
Ulcerative Colitis is an inflammatory disease of the bowel characterized
by chronic ulcers in the colon. The chief characteristic of this disorder is
bloody diarrhea. Colitis may involve only the left side of the colon or may
eventually extend to involve the entire bowel. However, in some cases it may
attack most of the large bowel simultaneously. The disease is usually
chronic, with repeated periods of exacerbation and remission. (For more
information on this disorder, choose "Ulcerative Colitis" as your search term
in the Rare Disease Database).
Therapies: Standard
Treatment of GVHD usually consists of glucocorticoid (steroid) drugs and a
combination of cyclosporine (Sandimmune) and methotrexate. In some cases
where GVHD is resistant to steroid drugs, treatment with anti-interleukin-2
receptor monoclonal antibody has been used. Treatment is often given to
patients before a blood transfusion or bone marrow transplant, or the blood
may be treated before being given to the recipient. These methods of pre-
treatment often keep GVHD from developing.
Therapies: Investigational
The treatment of GVHD patients with the orphan drug Thalidomide is being
tested in cases where the patient is unable to tolerate steroids or has a
poor response to them. However, Thalidomide can have severe side effects on
a developing fetus if the patient is pregnant, so extreme care must be taken
in choosing persons to receive this form of treatment. Dr. Georgia B.
Vogelsang, Assistant Professor of Oncology at Johns Hopkins Medical
Institutions, Baltimore, MD received an FDA orphan drug grant to study the
immunosuppressive properties of Thalidomide as a first line therapy in
patients with GVHD and other serious diseases. Further investigation is
needed to determine the long-term safety and effectiveness of this treatment.
The drug is manufactured by:
Andrulis Research Corp.
4600 East West Highway, Suite 900
Bethesda, MD 20814
(301) 657-1700
Thalidomide is available in England under special license from Penn
Pharmaceuticals of Tredegar, South Wales.
Interleukin-1 Antagonist, Human Recombinant (Antril) is being sponsored
by Synergen, Inc., Boulder, CO, as an investigational therapy for GVHD in
transplant patients.
The orphan drug humanized anti-tac is being tested as a treatment to
prevent Graft vs Host Disease following bone marrow transplantation. The
drug is sponsored by Hoffmann-La Roche of Nutley, NJ.
This disease entry is based upon medical information available through
April 1993. 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 Graft vs Host Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Caitlin Raymond International Registry of Bone Marrow Donor Banks
University of MA Medical Center
55 Lake Ave
Worcester, MA 01655
NIH/National Heart, Lung and Blood Institute (NHLBI)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4236
References
CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp.950, 1040.
GASTROINTESTINAL INFLAMMATION AFTER BONE MARROW TRANSPLANTATION: GRAFT-
VS-HOST DISEASE OR OPPORTUNISTIC INFECTION?, B. Jones, et al.; AJR Am J
Roentgenol, (February, 1988, issue 150 (2)). Pp. 277-281.
TRANSFUSION-ASSOCIATED GRAFT-VS-HOST DISEASE IN PATIENTS WITH
MALIGNANCIES. REPORT OF TWO CASES AND REVIEW OF THE LITERATURE, S.D.
Decoste, et al.; Arch Dermatol, (October, 1990, issue 126 (10)). Pp. 1324-
1329.
A RETROSPECTIVE ANALYSIS OF THERAPY FOR ACUTE GRAFT-VS-HOST DISEASE:
INITIAL TREATMENT. P.J. Martin, et al.; Blood, (October 15, 1990, issue 76
(8)). Pp. 1464-1472.