title: Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia.
abstract: 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.
authors.affiliation: Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.
authors.forename: E
authors.initials: E
authors.lastname: Matutes
authors.forename: V
authors.initials: V
authors.lastname: Brito-Babapulle
authors.forename: J
authors.initials: J
authors.lastname: Swansbury
authors.forename: J
authors.initials: J
authors.lastname: Ellis
authors.forename: R
authors.initials: R
authors.lastname: Morilla
authors.forename: C
authors.initials: C
authors.lastname: Dearden
authors.forename: A
authors.initials: A
authors.lastname: Sempere
authors.forename: D
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D002869:Chromosome Aberrations; D002883:Chromosomes, Human, Pair 14; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D015463:Leukemia, Prolymphocytic / Q000235:genetics / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D015649:Pentostatin / Q000627:therapeutic use; D011379:Prognosis; D012074:Remission Induction; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology
chemical_list: D015703:Antigens, CD; D015649:Pentostatin