Esophageal graft-versus-host disease.
A 61-year-old man with a history of acute myeloid leukemia, stem cell transplant, and graft-versus-host disease (GVHD) presented with solid food dysphagia. Flexible endoscopic evaluation of swallowing was normal, and a barium esophagram showed nonspecific dysmotility. Transnasal esophagoscopy demonstrated diffuse narrowing of the esophagus, proximal esophageal stricture, irregular squamocolumnar junction, and white plaques throughout the mucosa (figure). Pathology showed crypt dropout of the gastric glands, consistent with mild GVHD.
Treatment for this patient's mild GVHD included continued systemic immunosuppression with prednisone as well as local therapy. Targeting his esophageal dysmotility, a home dysphagia exercise regimen and a proton pump inhibitor for reflux were recommended. Trial discontinuation of steroids led to worsening dysphagia and recurrent esophageal stricture. The patient's symptoms were ultimately controlled with immunosuppression by steroids and tacrolimus, and he was then able to tolerate a soft diet.
GVHD occurs following allogeneic hematopoietic-cell transplantation; the chronic form usually occurs within the first 3 years. The exact pathophysiology of chronic GVHD is incompletely understood, although poor T-cell regulation is thought to play a central role. (1) Multiple organ systems may be involved. Distinguishing between acute and chronic GVHD is not solely dependent on time of development since transplant. One distinguishing feature of chronic GVHD sufficient for diagnosis is the presence of an esophageal web or stricture of the upper to mid-third of the esophagus. (2)
Grading of chronic GVHD uses both the number of organ systems involved as well as the level of disability caused by symptoms. Moderate and severe GVHD may require systemic therapy with immunosuppressants. (2) Esophageal involvement will likely present with dysphagia, for solids more so than liquids, due to the stricture or web. A barium esophagram may reveal nonspecific dysmotility or stricture. Diagnosis is assisted by direct visualization of the esophagus and biopsy of any lesions.
Histopathologic findings include inflammatory cell (lymphocytic and eosinophilic) infiltration and apoptotic features. (3) Treatment for esophageal symptoms includes dilation, topical glucocorticoid therapy, and systemic immunosuppression.
(1.) Dutt S, Tseng D, Ermann J, et al. Naive and memory T cells induce different types of graft-versus-host disease. J Immunol 2007;179 (10):6547-54.
(2.) Filipovich AH, Weisdorf D, Pavletic S, et al. National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group report. Biol Blood Marrow Transplant 2005;11 (12):945-56.
(3.) Daneshpouy M, Socie G, Lemann M, et al. Activated eosinophils in upper gastrointestinal tract of patients with graft-versus-host disease. Blood 2002;99(8):3033-40.
Jeanne L. Hatcher, MD; S. Carter Wright, MD; Catherine Rees Lintzenich, MD, FACS
From the Department of Otolaryngology, Wake Forest School of Medicine, Winston-Salem, N.C.
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|Title Annotation:||DYSPHAGIA CLINIC|
|Author:||Hatcher, Jeanne L.; Wright, S. Carter; Lintzenich, Catherine Rees|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Case study|
|Date:||Feb 1, 2013|
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