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

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.

• Authors: R Pawson, E Matutes, V Brito-Babapulle, H Maljaie, M Hedges, J Mercieca, M Dyer, D Catovsky
• Journal: Leukemia (Leukemia) Vol. 11 Issue 7 Pg. 1009-13 (Jul 1997) ISSN: 0887-6924 [Print] England
• PMID: 9204983 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

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