Fight with the gastro-chameleon – celiac disease patient with hematology symptoms

Introduction: Celiac disease is an immune-mediated disorder of the small intestinal mucosa that may result a variety of clinical symptoms and complications. Celiac disease is a true “gastro-chameleon“ due to the highly variable extra intestinal and atypical clinical presentations of the disease. Celiac studies have indicated that there is a 30 to 40-fold increased risk of intestinal lymphoma. In contrary, unrecognized forms may result in generalized lymphadenopathy which could show the clinical picture of non-Hodgkin lymphoma.

Case report: 44-year-old white male who presented with flatulence, weight loss, abdominal cramps, papulous skin lesions and edema was investigated in the Hematology Department. Iron deficiency, anemia (Hgb 89g/l),low level of albumin, cholesterol with elevated ESR,CRP and liver functions were detected.

History: Hepato-splenomegaly with pathologic abdominal lymph nodes on abdominal US and CT with the same clinical findings in 2007, but no appearance since that. Controlled CT findings were similar with progression: small bowel dilation, jejunization of the ileum, mesenteric and para-aortic lymph node conglomerates, hepato-splenomegaly with ascites. Suggested diagnosis was enteropathy associated T-cell lymphoma (EATL). Due to the known risk of ETL in celiac disease, duodenum biopsies were taken. Pathology report showed high degree of crypt hyperplasia and subtotal villous atrophy – Marsh 3 stage.

Serology: Antihuman tissue transglutaminase (TTG) levels: IgG 57 U/ml, IgA >200 U/ml (0–10). Bone marrow biopsy: reactive proliferate hemopoesis, no signs of lymphoid infiltration. Pathology report of mesenteric lymph node of laparotomy: reactive lymph node with sinus histiocytosis Finally the diagnosis of gluten sensitive enteropathy could be made. Six months later, normal lab. and histology parameters with significant regression of CT scan detected following gluten-free diet. Patient gained 12kg. We would like to emphasize that the recognition of atypical features of celiac disease could save the petient from chemotherapy and made him curable only with diet.