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Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia
E Matutes, V Brito-Babapulle, J Swansbury, J Ellis, R Morilla, C Dearden, A Sempere and D Catovsky
Academic Department of Haematology and Cytogenetics, Royal Marsden
Hospital, London, UK.
We describe the clinical and laboratory findings of 78 adult patients with
T-prolymphocytic leukemia (T-PLL) studied over the last 12 years. The main
disease features were splenomegaly (73%), lymphadenopathy (53%),
hepatomegaly (40%), skin lesions (27%), and a high leukocyte count (greater
than 100 x 10(9)/L in 75%) with nucleolated prolymphocytes. A variant form
with small, less typical cells was recognized in 19%. Membrane markers
defined a postthymic phenotype TdT- , CD2+, CD3+, CD5+, CD7+; in 65%, the
cells were CD4+ CD8-, in 21%, they coexpressed CD4 and CD8, and, in 13%,
they were CD4- CD8+. Serology for human T-cell leukemia/lymphoma virus
Type-I (HTLV-I) was negative in the 27 cases investigated. Cytogenetic
analysis in 30 cases showed a consistent abnormality of chromosome 14,
usually inv (14), with breakpoints at q11 and q32 in 76% of cases. Trisomy
8, including iso8q, was shown in 53%; t (11;14)(q13;q32) was documented in
one case; and one had a normal karyotype. The clinical course was
progressive with a median survival of 7.5 months. Thirty-one patients were
treated with 2' deoxycoformycin and 15 responded (3 complete remissions and
12 partial remissions); the response rate (48%) increased to 58% in
patients with a CD4+ CD8- phenotype. The median survival of responders was
16 months and of nonresponders 10 months; other treatments were less
effective. T-PLL is a distinct clinico-pathologic entity with aggressive
course and characteristic chromosome abnormalities. A subgroup of patients
may benefit from deoxycoformycin.
Volume 78,
Issue 12,
pp. 3269-3274,
12/15/1991
Copyright © 1991 by The American Society of Hematology
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