Large granular lymphocytic leukemia: a treatable form of refractory celiac disease.

Malamut, Georgia; Meresse, Bertrand; Verkarre, Virginie; Kaltenbach, Sophie; Montcuquet, Nicolas; Duong Van Huyen, Jean-Paul; Callens, Céline; Lenglet, Julien et al. · Gastroenterology · 2012

case_report · Level V

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Abstract

Large granular lymphocyte leukemia (LGL) is characterized by clonal expansion of CD3+ T cells or CD3(-) natural killer cells and frequently is associated with autoimmune diseases. We describe 2 patients with celiac disease who no longer responded to gluten-free diets after they developed T-cell LGL, with intestinal localization of malignant lymphocytes. Flow cytometry phenotyping of isolated intestinal intraepithelial and lamina propria cells eliminated type II refractory celiac disease, identifying large-sized CD8(+)CD57(+) T cells. Treatment with a combination of cyclosporine and methotrexate restored the patients' sensitivity to gluten-free diets. LGL therefore might be a cause of refractory celiac disease that is sensitive to immunosuppressive therapy.

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