title: Gamma heavy chain disease associated with large granular lymphocytic leukemia: A report of two cases and review of the literature.
abstract: OBJECTIVE
  Gamma heavy chain diseases (γHCD) and large granular lymphocyte (LGL) leukemia are two rare lymphoproliferative diseases, respectively with B and T phenotype. Both γHCD and LGL leukemia share some similar clinical features, such as cytopenias, splenomegaly, and recurrent infections. Association of these two diseases is exceptional and suggest pathogenic link. We report two cases of γHCD associated with T-LGL leukemia.
  
  
  METHODS
  Patient 1 was a 70-year-old woman, with lymphoplasmacytic lymphoma, refractory to chlorambucil-rituximab treatment. She developed during the follow up a γHCD with T-LGL leukemia, unresponsive to melphalan, thalidomide, and steroids, requiring supportive care. Patient 2 was a 40-year-old man with chronic severe asymptomatic neutropenia, revealing both γHCD and T-LGL leukemia. He is still well without any treatment nor complications, with 7 years follow up.
  
  
  CONCLUSIONS
  Several types of B lymphoproliferative disease are associated with LGL leukemia. Although exceptional, this association of two rare lymphoproliferative disorders, with a different phenotype, does not seem fortuitous.
authors.affiliation: a Service de Médecine Interne, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Anais
authors.initials: A
authors.lastname: Wahbi
authors.affiliation: a Service de Médecine Interne, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Neel
authors.affiliation: a Service de Médecine Interne, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Francois
authors.initials: F
authors.lastname: Perrin
authors.affiliation: a Service de Médecine Interne, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Julie
authors.initials: J
authors.lastname: Graveleau
authors.affiliation: b Service d'Hématologie, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Beatrice
authors.initials: B
authors.lastname: Mahe
authors.affiliation: c Service de Biochimie, CHU Hôtel Dieu , 1 Place Alexis-Ricordeau, 44093 Nantes Cedex 1 , France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Dejoie
authors.affiliation: a Service de Médecine Interne, CHU Hôtel Dieu, 1 Place Alexis-Ricordeau , 44093 Nantes Cedex 1 , France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006362:Heavy Chain Disease / Q000150:complications / Q000188:drug therapy; D006801:Humans; D007945:Leukemia, Lymphoid / Q000150:complications / Q000188:drug therapy; D008297:Male
keywords: Franklin disease; Gamma heavy chain disease; Large granular lymphocyte leukemia