Chronic GvHD

Abstract

Graft-versus-host disease (GvHD) is one of the most common complications seen after allogeneic hematopoietic stem cell transplantation (HSCT) and can be life-threatening. The risk of GvHD increases as HLA disparity between the donor and recipient increases. Historically, GvHD has been divided into two major groups, acute and chronic, based on the time in the HSCT course that it was diagnosed. GvHD was considered acute if diagnosed prior to 100 days post-HSCT, whereas patients were diagnosed with chronic GvHD after 100 days post-HSCT regardless of the presentation. With the advent of alternative donor HSCT approaches, including umbilical cord blood transplantation (UCBT), the use of reduced-intensity conditioning regimens, and donor lymphocyte infusion(s) after HSCT, it has become apparent that GvHD needs to be categorized based on its characteristics and not strictly by the timeline of presentation. While the management of both acute and chronic GvHD is based on immunosuppression mostly directed at T-cells, the approach to treatment and monitoring is different. Acute and chronic GvHD have overlap in their target organs (i.e., skin, liver, GI tract, and lungs). However, their effect on these target organs differs. In addition, chronic GvHD may affect every organ system, although most have 1-3 organ systems involved. Chronic GvHD acts like an autoimmune disease and often is treated as such, with corticosteroids considered first-line treatment. Because of the natural history as well as treatment with immunosuppression of chronic GvHD, patients with chronic GvHD are very vulnerable to infection, particularly opportunistic and life-threatening, and infection is the leading cause of death of patients with chronic GvHD. Management of chronic GvHD requires a multidisciplinary team approach; early recognition along with appropriate, comprehensive intervention and supportive care will help curtail longterm complications and disability. This chapter addresses the risk factors, clinical features, diagnostic criteria, classification, and the management of chronic GvHD. Chapter 18 discusses acute GvHD.