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Clinical manifestations, diagnosis, and grading 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'.)

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

EPIDEMIOLOGY

Chronic graft-verus-host disease (GVHD) can occur after previous or ongoing acute GVHD or in patients without a history of acute GVHD (eg, de novo disease). The exact incidence of chronic GVHD after allogeneic hematopoietic cell transplant (HCT) is unknown. While chronic GVHD occurs in approximately 40 percent of allogeneic HCT recipient, reported incidence rates range from 6 to 80 percent, depending upon the presence of risk factors and the diagnostic criteria used [1-4]. While risk factors for the development of GVHD have been identified, reliable estimates of GVHD incidence in various cohorts are not available due to variability in the identification, measurement, and documentation of chronic GVHD.

                          

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Literature review current through: Nov 2016. | This topic last updated: Mon Aug 15 00:00:00 GMT+00:00 2016.
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