$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.