Graft versus Host Disease

Graft versus Host Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Graft versus Host Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • GVHD

Disorder Subdivisions

  • Acute GVHD
  • Chronic GVHD

General Discussion

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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Standard Therapies

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.

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, contact:

www.centerwatch.com

References

TEXTBOOKS

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996: 975.



JOURNAL ARTICLES

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

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

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Locks of Love

234 Southern Blvd.

West Palm Beach, FL 33405-3099

Tel: (561)833-7332

Fax: (561)833-7962

Tel: (888)896-1588

TDD: (561)833-7332

Email: info@locksoflove.org

Internet: http://www.locksoflove.org



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

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). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, 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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