Diagnosis and clinical management of chronic graft-versus-host disease

Chronic graft-versus-host disease (GVHD) occurs in approximately 60% of patients who survive for more than 100 days after receiving an allogeneic marrow or peripheral blood stem cell transplant without T-cell depletion of the graft. Chronic GVHD represents a major cause of morbidity and mortality among hematopoietic stem cell transplant recipients. Risk factors for the development of chronic GVHD and for mortality among patients who develop this complication have been defined, but the pathogenesis of chronic GVHD is not well understood. This review discusses the clinical manifestations that lead to a diagnosis of chronic GVHD and outlines an approach for therapy with glucocorticoids and extended administration of a calcineurin inhibitor. The judicious use of glucocorticoids at the lowest effective dose and alternate-day administration can minimize steroid-related side effects. Antibiotic prophylaxis to prevent infection and supportive care to minimize morbidity and prevent disability are critically important components in the management of patients with chronic GVHD. Approximately 50% of patients with chronic GVHD are able to discontinue immunosuppressive treatment within 5 years after the diagnosis, and 10% require continued treatment beyond 5 years. The remaining 40% die or develop recurrent malignancy before the chronic GVHD resolves. An improved understanding of the pathogenesis of the disease is needed to develop more effective therapy. (C) 2004 The Japanese Society of Hematology.