Large granular lymphocytic leukemia: a treatable form of refractory celiac disease.
Malamut G,Meresse B,Verkarre V,Kaltenbach S,Montcuquet N,Duong Van Huyen J,Callens C,Lenglet J,Rahmi G,Samaha E,Ranque B,Macintyre E,Radford-Weiss I,Hermine O,Cerf-Bensussan N,Cellier C
Source :
2012 Aug 23
Pmid / DOI:
22922421
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.Copyright © 2012 AGA Institute. Published by Elsevier Inc. All rights reserved.