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NORD is very grateful to Catharina Schuetz, MD, MSc, Ulm University, Department of Pediatrics (Immunology, BMT), Germany, for assistance in the preparation of this report.
Graft versus Host Disease (GVHD) is a rare disorder that can strike persons whose immune system is deficient or suppressed and who have received a bone marrow transplant or a nonirradiated blood transfusion. Symptoms may include skin rash, intestinal problems and liver dysfunction.
GVHD occurs most frequently after allogeneic bone marrow transplant and initially leads to dermatitis (a skin rash), gastrointestinal problems and liver dysfunction. In its chronic form involvement of mucosa (mouth and eyes) resembling sicca syndrome, lungs (resembling bronchiolitis obliterans) and the muskuloskeletal system (resembling myositis) is observed. GVHD affects about 60% of all bone marrow transplant but usually is limited and mild.
GVHD can be acute (sudden) or chronic (long lasting). Acute GVHD occurs in the first 100 days (at earliest 2 to 3 weeks) following bone marrow transplantation. The first symptoms are usually mild skin rash, liver dysfunction and intestinal problems. In some cases the patients may suddenly show very severe skin problems, diarrhea, nausea, abdominal pain and liver failure.
Chronic GVHD is used for GVHD lasting beyond 100 days and usually persists long after a bone marrow transplant. The signs and symptoms are similar to those of the acute GVHD, but in addition to the skin, intestinal and liver problems, chronic GVHD may also involve mucosa, lungs and the musculoskeletal system. Long term consequences may be scleroderma-like skin changes and bronchiolitis obliterans.
GVHD is caused by donor T cells recognizing foreign antigens (histocompatibility or human leucocyte antigens) on the recipient's cells and reacting to them. Prior to allogeneic bone marrow transplants recipients usually undergo myeloablative treatment with radiation or chemotherapy to destroy their own diseased bone marrow and weaken their immune system. When receiving the bone marrow or stem cell transplant, immunocompetent donor lymphocytes recognize foreign minor locus histocompatibility antigens on the recipient's cells resulting in GVHD.
GVHD affects males and females of all ages who have been immunosuppressed before being given a bone marrow transplant or a nonirradiated blood transfusion containing allogeneic lymphocytes. The risk of GVHD usually increases with the recipient's age and with the degree of HLA differences between donor and recipient unless fully T-cell depleted.
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 intial 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.
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.
Treatment of GVHD usually consists of immunosuppressive drugs including glucocorticoid (steroid) drugs and a combination of cyclosporine (Sandimmune) and methotrexate. Instead of cyclosporine other calcineurin inhibitors (tacrolimus) or an mTOR inhibitor (sirolimus) may be chosen. In some cases where GVHD is resistant to the above treatments antithymocyte globulin (ATG) may by used. Prevention of GVHD consists of prophylactic treatment prior to bone marrow transplant, mostly using cyclosporine and T-cell depletion of the graft. Blood may be treated by radiation before being given to the recipient in order to suppress the donor's lymphocytes. These prophylactic measures often keep GVHD from developing.
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Report last updated: 2009/01/14 00:00:00 GMT+0