Gamma Heavy Chain Disease with T-cell Large Granular Lymphocytic Leukemia: A Case Report and Review of the Literature

Intern Med. 2016;55(4):399-403. doi: 10.2169/internalmedicine.55.5042. Epub 2016 Feb 15.

Abstract

Gamma heavy chain disease (gHCD) is a rare lymphoproliferative disorder characterized by the production of a truncated immunoglobulin heavy chain. Although some cases of gHCD are concurrent with other lymphoid neoplasms, few have been reported. We herein present the case of a 73-year-old woman with gHCD and T-cell large granular lymphocytic leukemia. A multiparameter flow cytometry analysis revealed neoplastic cells that were positive for CD28, a marker of T-cell activation, the anti-apoptotic antigen of neoplastic plasma cells, CD38 and CD45. The results of this multiparameter flow cytometry analysis may contribute to furthering the understanding of the clinicopathological features of gHCD.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Anemia / etiology
  • Anemia / immunology*
  • Cytogenetic Analysis
  • Fatigue / etiology
  • Fatigue / immunology*
  • Female
  • Flow Cytometry
  • Heavy Chain Disease / complications
  • Heavy Chain Disease / immunology
  • Heavy Chain Disease / pathology*
  • Humans
  • Immunoglobulin Heavy Chains
  • Immunoglobulin gamma-Chains / blood
  • Immunoglobulin gamma-Chains / metabolism*
  • Leukemia, Large Granular Lymphocytic / complications
  • Leukemia, Large Granular Lymphocytic / immunology
  • Leukemia, Large Granular Lymphocytic / pathology*
  • Lymphocytes / metabolism*

Substances

  • Immunoglobulin Heavy Chains
  • Immunoglobulin gamma-Chains