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Graft versus Host Disease
National Organization for Rare Disorders, Inc.
Synonyms
Disorder Subdivisions
General Discussion
Graft versus 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 versus 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 Populations
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 versus 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.)
Standard Therapies
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.
Investigational Therapies
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222 TTY: (866) 411-1010 Email: prpl@cc.nih.gov
For information about clinical trials sponsored by private sources, in the main, contact: www.centerwatch.com
References
Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996: 975.
Jacobsohn DA, Novel therapeutics for the treatment of graft-versus-host disease. Expert Opin Investig Drugs. 2002;11:1271-80.
Carpenter PA, et al., A humanized non-FcR-binding anti-CD3 antibody, visilizumab, for treatment of steroid-refractory acute graft-versus-host disease. Blood. 2002;99:2712-9.
Rivkina AM, Stump LS, Infliximab in graft-versus-host disease. Am J Health Syst Pharm. 2002;59:1271-5.
Deeg HJ, et al., Treatment of steroid-refractory acute graft-versus-host disease with antibody-CD147 monoclonal antibody ABX-CBL. Blood. 2001;98:2052-8.
Greinix HT, et al., Extracorporeal photochemotherapy in the treatment of severe steroid-refractory acute graft-versus-host disease: a pilot study. Blood. 2000;96:2426-31.
McDonald GB, et al., Oral beclomethasone dipropionate for treatment of intestinal graft-versus-host disease: a radonmized, controlled trial. Gastroenterology. 1998;115:28-35. Comment In: Gastroenterology. 1998;115:220-2.
Zic JA, et al., The North American experience with photopheresis. Ther Apher. 1999;3:50-62.
Bonnie C, et al., HSV-TV gene transfer into donor lymphocytes for control of allogeneic graft-versus-leukemia. Science. 1997;276:1719-24.
Chao NJ, et al., Cyclosporine, methotrexate, and prednisone compared with cyclosporine and prednisone for prophylaxis of acute graft-versus-host disease. N Engl J Med. 1993;329:1225-30.
Decoste AD, et al., Transfusion-associated graft-vs-host disease in patients with malignancies. Report of two cases and review of the literature. Arch Dermatol. 1990;126:1324-29.
Martin PJ, et al., A retrospective analysis of therapy for acute graft-vs-host disease: initial treatment. Blood. 1990;76:1464-72.
Jones B, et al., Gastrointestinal inflammation after bone marrow transplantation: graft- vs-host disease or opportunistic infection? AJR Am J Roentgenol. 1988;150:277-81.
Resources
Caitlin Raymond International Registry
UMASS Memorial Medical Center 55 Lake Avenue North Worcester, MA 01655 Tel: (508)334-8969 Fax: (508)334-8972 Tel: (800)726-2824 Email: info@CRIR.org Internet: http://www.crir.org
NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105 Bethesda, MD 20824-0105 Tel: (301)592-8573 Fax: (301)251-1223 Email: nhlbiinfo@rover.nhlbi.nih.gov
Locks of Love
2925 10th Ave North Suite 102 Lake Worth, FL 33461 Tel: (561)963-1677 Fax: (561)963-9914 Tel: (888)896-1588 TDD: (561)963-1677 Email: info@locksoflove.org Internet: http://www.locksoflove.org
Autoimmune Information Network, Inc
PO Box 4121 Brick, NJ 08723 Tel: (732)262-0450 Fax: (732)262-0450 Email: autoimmunehelp@aol.com Internet: http://www.aininc.org
European Society for Immunodeficiencies (ESID)
c/o Dr. Esther de Vries Jeroen Bosch Hospital Dept. Paediatrics P.O. Box 90153 Hertogenbosch, 5200 ME's Netherlands Tel: +31 73-6992965 Fax: +31 73-6992948 Email: info@esid.org Internet: http://www.esid.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.
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listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Database completely current and accurate. Please check with the agencies listed in the Resources section
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Last Updated: 3/14/2008
Copyright 1991, 1992, 1993, 1997, 1998, 1999, 2000, 2001, 2002
National Organization for Rare Disorders, Inc.
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