Mucosal Chronic GVHD Affecting the Oral Pharyngeal, Esophageal, and Anogenital Regions
Chronic GVHD (cGVHD) targets several regions not affected by acute GVHD (aGVHD), including the oropharynx, the upper portion of the esophagus, and the anogenital region. These regions are surfaced by nonkeratinized squamous mucosa and have similar histolo
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Howard M. Shulman, David M. Hockenbery, and Cecilia C. S. Yeung
Clinical History Eight months after Hodgkin lymphoma recurred following an autologous HSCT, this 30-year-old woman received an HLA-matched unrelated donor allogeneic transplant. Hyperacute GVHD developed soon afterward. By day 95, she had developed chronic GVHD (cGVHD) involving multiple sites, including the skin with focal lichenoid skin changes, oral mucositis (Figs. 12.1, 12.2, and 12.3), keratoconjunctivitis sicca syndrome, vaginitis, and pulmonary bronchiolitis obliterans syndrome. She responded well to IS but had persistent oral and
H. M. Shulman (*) · C. C. S. Yeung Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Department of Pathology, University of Washington School of Medicine, Seattle, WA, USA Pathology Section, Seattle Cancer Care Alliance, Seattle, WA, USA e-mail: [email protected]; [email protected] D. M. Hockenbery Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Human Biology Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Public Health Sciences Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Division of Gastroenterology, University of Washington School of Medicine, Seattle, WA, USA e-mail: [email protected] © Springer Nature Switzerland AG 2019 C. C. S. Yeung, H. M. Shulman (eds.), Pathology of Graft vs. Host Disease, https://doi.org/10.1007/978-3-319-42099-8_12
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144 Fig. 12.1 Gross image of oral mucosa 4 years post-transplant. The erythematous extended tongue has atrophy of the papillae, and the facial skin has poikiloderma
Fig. 12.2 The gums and lip have a reticulated, erythematous appearance
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Fig. 12.3 An oral labial biopsy whose squamous mucosa has apoptotic change and chronic inflammation in the underlying submucosa
vaginal involvement (Fig. 12.4). At 15 months, she developed dysphagia with difficulty swallowing pills or dry food, but an upper esophagogastroduodenoscopy (EGD) was negative for GVHD. At 4 years (day 1461), EGD revealed mid-esophageal web formation (Figs. 12.5 and 12.6), and corresponding biopsy of her esophagus and stomach demonstrated GVHD (Fig. 12.7). Treatment with beclomethasone in corn oil alleviated the dysphagia. At 9 years posttransplant, the patient had persistent dysphagia with difficulty swallowing food, oral lichenoid lesions, severe ocular sicca requiring scleral lens, and some vaginal fibrosis. Her pulmonary status was stable but abnormal with mild to moderate dyspnea on exertion with an FEV1 of 46%.
Diagnosis Generalized cGVHD of mucosa with chronic inflammation and apoptosis involving (a) the oral cavity along with keratoconjunctivitis sicca; (b) upper esophagus with desquamation, narrowing, and fibrous web formation; and (c) anogenital region with vulvar lichen sclerosis et atrophicus with vaginal fibrosis/stenosis
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Fig. 12.4 Day 368 of severe cGVHD of the anal-genital region demonstr
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