Blastic NK-cell lymphoma/leukemia with T-cell receptor gamma rearrangement

Ann Hematol. 2001 Dec;80(12):752-4. doi: 10.1007/s00277-001-0380-0. Epub 2001 Oct 18.

Abstract

A 79-year-old Japanese man was admitted to our hospital with dyspnea in June 1999. Physical examination revealed general exanthema, hepatosplenomegaly, and lymphadenopathy. Increased numbers of abnormal cells were observed in peripheral blood; these cells were of lymphoblastic morphology with high nuclear/cytoplasm ratios and few azurophilic granules. Immunophenotypic analysis revealed positivity for CD2, CD4, CD56, and HLA-DR, and negativity for CD3, CD13, CD16, CD33, CD34, and T cell receptor (TCR). On genotypic analysis, TCRgamma chain was rearranged, but neither the TCRbeta chain nor TCRdelta chain. Despite an initial good response to chemotherapy the disease relapsed in the early stage, and the patient died 6 months after diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antigens, CD / analysis
  • Biopsy
  • Bone Marrow / pathology
  • C-Reactive Protein / analysis
  • Cell Nucleus / pathology
  • Cytoplasm / pathology
  • Erythema / pathology
  • Fatal Outcome
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor*
  • HLA-DR Antigens / analysis
  • Humans
  • Immunophenotyping
  • Killer Cells, Natural* / immunology
  • L-Lactate Dehydrogenase / blood
  • Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis*
  • Leukemia, Lymphocytic, Chronic, B-Cell / genetics
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology
  • Leukemia, Lymphoid / diagnosis*
  • Leukemia, Lymphoid / genetics
  • Leukemia, Lymphoid / pathology
  • Male
  • Receptors, Interleukin-2 / analysis
  • Skin / pathology

Substances

  • Antigens, CD
  • HLA-DR Antigens
  • Receptors, Interleukin-2
  • C-Reactive Protein
  • L-Lactate Dehydrogenase