title: Large granular lymphocytic leukemia: a treatable form of refractory celiac disease.
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.
authors.affiliation: Université Paris Descartes, Paris, France. georgia.malamut@egp.aphp.fr
authors.forename: Georgia
authors.initials: G
authors.lastname: Malamut
authors.forename: Bertrand
authors.initials: B
authors.lastname: Meresse
authors.forename: Virginie
authors.initials: V
authors.lastname: Verkarre
authors.forename: Sophie
authors.initials: S
authors.lastname: Kaltenbach
authors.forename: Nicolas
authors.initials: N
authors.lastname: Montcuquet
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Duong Van Huyen
authors.forename: Céline
authors.initials: C
authors.lastname: Callens
authors.forename: Julien
authors.initials: J
authors.lastname: Lenglet
authors.forename: Gabriel
authors.initials: G
authors.lastname: Rahmi
authors.forename: Elia
authors.initials: E
authors.lastname: Samaha
authors.forename: Brigitte
authors.initials: B
authors.lastname: Ranque
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Macintyre
authors.forename: Isabelle
authors.initials: I
authors.lastname: Radford-Weiss
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Nadine
authors.initials: N
authors.lastname: Cerf-Bensussan
authors.forename: Christophe
authors.initials: C
authors.lastname: Cellier
mesh_terms: D000368:Aged; D002446:Celiac Disease / Q000178:diet therapy* / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use; D055050:Diet, Gluten-Free; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D054066:Leukemia, Large Granular Lymphocytic / Q000188:drug therapy* / Q000473:pathology; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008727:Methotrexate