Sezary cell leukaemia: a distinct T cell disorder or a variant form of T prolymphocytic leukaemia?
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.
Pawson
R
R
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.
Matutes
E
E
Brito-Babapulle
V
V
Maljaie
H
H
Hedges
M
M
Mercieca
J
J
Dyer
M
M
Catovsky
D
D
D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D015458:Leukemia, T-Cell / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012751:Sezary Syndrome / Q000175:diagnosis* / Q000473:pathology