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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.
-
-