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

Gastroenterology. 2012 Dec;143(6):1470-1472.e2. doi: 10.1053/j.gastro.2012.08.028. Epub 2012 Aug 23.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Celiac Disease / diet therapy*
  • Celiac Disease / pathology
  • Cyclosporine / therapeutic use
  • Diet, Gluten-Free*
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Leukemia, Large Granular Lymphocytic / drug therapy*
  • Leukemia, Large Granular Lymphocytic / pathology
  • Methotrexate / therapeutic use
  • Middle Aged
  • Treatment Failure
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Cyclosporine
  • Methotrexate