Sezary cell leukaemia: a distinct T cell disorder or a variant form of T prolymphocytic leukaemia?

Leukemia. 1997 Jul;11(7):1009-13. doi: 10.1038/sj.leu.2400710.

Abstract

We report the clinical, ultrastructural, immunophenotypic and virological features of nine cases of a rare type of mature T cell disorder formerly designated Sezary cell leukaemia. All patients presented with lymphocytosis ranging from 12.7 to 133 x 10(9)/l, bone marrow infiltration, splenomegaly and lymphadenopathy. Skin involvement was absent at presentation but developed as a terminal event in two patients, one of whom showed a pattern of dermal infiltration different from that characteristic of Sezary syndrome. Cells from eight cases bore a mature T cell phenotype and electronmicroscopy revealed lymphocytes with cerebriform nuclei resembling Sezary cells. All cases except one were HTLV-I negative. Patients were treated with various chemotherapy regimens but with poor outcome, the median survival being 13 months. Laboratory and clinical data suggest great similarity between Sezary cell leukaemia and T prolymphocytic leukaemia (T-PLL), namely coexpression of CD4 and CD8 (3/9 cases), identical chromosomal abnormalities in the three cases studied (isochromosome 8q plus inversion 14 or t(X;14)(q28;q11)) and a remarkable sensitivity to CAMPATH-1H (complete remission of 21 months' duration in one patient), suggesting that this entity could be considered a variant form of T-PLL. The alternative diagnosis of adult T cell leukaemia/lymphoma could not be excluded in one patient in whom positive HTLV-I serology was documented.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Diagnosis, Differential
  • Female
  • Humans
  • Leukemia, Prolymphocytic / diagnosis*
  • Leukemia, Prolymphocytic / immunology
  • Leukemia, Prolymphocytic / pathology
  • Leukemia, T-Cell / diagnosis*
  • Leukemia, T-Cell / immunology
  • Leukemia, T-Cell / pathology
  • Male
  • Middle Aged
  • Sezary Syndrome / diagnosis*
  • Sezary Syndrome / pathology