Gammadelta T-cell large granular lymphocyte (LGL) leukemia with spontaneous remission

Am J Hematol. 2004 Mar;75(3):168-72. doi: 10.1002/ajh.10480.

Abstract

T-cell large granular lymphocyte (LGL) leukemia is a clonal disorder with an indolent clinical course. In July 1995, a 46-year-old Japanese man was admitted to our hospital because his anemia had progressed. He had a white blood cell count of 3.9 x 10(9)/L with 75% lymphocytes, which were intermediate to large and had almost round nuclei and azurophilic granules, and anemia with a red blood cell count (RBC) of 2.69 x 10(12)/L, hemoglobin (Hb) of 9.5 g/dL, and hematocrit (Hct) of 28.3%. Electron microscopic examination showed that most of the lymphocytes had a parallel tubular array and dense core granules in their cytoplasm. Flow cytometry and Southern blotting of the T-cell antigen receptor (TCR) genes using the peripheral blood species showed monoclonal proliferation of LGLs with a CD3+, TCRgammadelta+, CD4-, CD8-, CD16+, CD56-, CD57-, HLA-DR+ phenotype, and a TCR gamma gene rearrangement, respectively, suggesting that the patient was diagnosed as having gammadelta T-cell LGL leukemia. He had no symptoms, organomegaly, or skin lesions. About 1.5 years after diagnosis, the anemia gradually improved with disappearance and appearance of a rearranged band in the TCR-gamma gene and TCR-beta gene, respectively. About 7 years after diagnosis, the anemia improved completely with a RBC of 5.01 x 10(12)/L, Hb of 14.8 g/dL, and Hct of 44.3%, and he was in complete remission without TCR-beta and -gamma gene rearrangements. He had received no therapy. This is the first report of spontaneous remission of gammadelta T-cell LGL leukemia.

Publication types

  • Review

MeSH terms

  • Blotting, Southern
  • Gene Rearrangement, T-Lymphocyte
  • Humans
  • Leukemia, T-Cell / genetics
  • Leukemia, T-Cell / pathology*
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Receptors, Antigen, T-Cell, gamma-delta / genetics*
  • Remission, Spontaneous
  • T-Lymphocyte Subsets

Substances

  • Receptors, Antigen, T-Cell, gamma-delta