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Treatment of chronic graft-versus-host disease

Author
Nelson J Chao, MD
Section Editor
Robert S Negrin, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Acute and chronic graft-verus-host disease (GVHD) are multisystem disorders that are common complications of allogeneic hematopoietic cell transplant (HCT). GVHD occurs when immune cells transplanted from a non-identical donor (the graft) recognize the transplant recipient (the host) as foreign, thereby initiating an immune reaction that causes disease in the transplant recipient. (See "Pathogenesis of graft-versus-host disease".)

GVHD has been classically divided into acute and chronic variants based upon the time of onset using a cutoff of 100 days. However, this conventional division has been challenged by the recognition that signs of acute and chronic GVHD may occur outside of these designated periods. This observation has led to the increased use of clinical findings, rather than a set time period, to differentiate between acute and chronic GVHD. The widely accepted National Institutes of Health (NIH) consensus criteria for the diagnosis of GVHD include an overlap syndrome in which diagnostic or distinctive features of chronic GVHD and acute GVHD appear together [1].

Clinical manifestations of chronic GVHD include skin involvement resembling lichen planus or the cutaneous manifestations of scleroderma; dry oral mucosa with ulcerations and sclerosis of the gastrointestinal tract; and a rising serum bilirubin concentration. In contrast, patients with acute GVHD commonly demonstrate a classic maculopapular rash; abdominal cramps with diarrhea; and a rising serum bilirubin concentration. (See "Clinical manifestations, diagnosis, and grading of acute graft-versus-host disease", section on 'Clinical and histological manifestations'.)

Chronic GVHD is the single major factor determining long-term quality of life following HCT. Because of the profound immunosuppression observed with this disorder, recurrent infections occur in almost all affected patients. These complications account for most of the morbidity and mortality associated with chronic GVHD. (See "Quality of life following hematopoietic cell transplantation", section on 'Chronic graft-versus-host disease'.)

This topic review will discuss the treatment of chronic GVHD. The diagnosis of chronic GVHD and the prevention and treatment of acute GVHD are discussed separately. (See "Clinical manifestations, diagnosis, and grading of chronic graft-versus-host disease" and "Prevention of acute graft-versus-host disease" and "Treatment of acute graft-versus-host disease".)

                            

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Literature review current through: Nov 2016. | This topic last updated: Mon Sep 19 00:00:00 GMT 2016.
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