title: Indolent course as a relatively frequent presentation in T-prolymphocytic leukaemia. Groupe Français d'Hématologie Cellulaire.
abstract: T-prolymphocytic leukaemia (T-PLL) is a rare disorder with a poor outcome. Presentation features were studied in 78 T-PLL cases. Although 53 patients (group A) presented with typical progressive disease including rapidly increasing leucocytosis. 25 patients (group B) experienced an initial indolent clinical course with stable moderate leucocytosis. The morphology and antigenic profile of abnormal cells were similar in both groups, except for a lower incidence of CD45RO+ CD45RA- pattern in group B. A high incidence of inv(14)(q11;q32), t(14;14)(q11;q32) and i(8)(q10) chromosomal abnormalities were found in both groups. After an initial indolent phase (median 33 months; 6-103 months), 16 group B patients progressed to an aggressive stage with clinical and laboratory features similar to group A. Moreover, median survival after progression was short in both groups. In conclusion, T-PLL may start as an indolent disease similar to that reported in ataxia telangectasia. In this rare genetic disorder, some patients develop stable T-cell clones which progress toward T-PLL-like leukaemia. Moreover, ATM gene mutations have been reported in T-PLL. Thus, both diseases are likely to be closely related.
authors.affiliation: Laboratoire d'Hématologie, Centre Hospitalier Universitaire de Nantes, France.
authors.forename: R
authors.initials: R
authors.lastname: Garand
authors.forename: J
authors.initials: J
authors.lastname: Goasguen
authors.forename: A
authors.initials: A
authors.lastname: Brizard
authors.forename: J
authors.initials: J
authors.lastname: Buisine
authors.forename: A
authors.initials: A
authors.lastname: Charpentier
authors.forename: J F
authors.initials: JF
authors.lastname: Claisse
authors.forename: E
authors.initials: E
authors.lastname: Duchayne
authors.forename: M
authors.initials: M
authors.lastname: Lagrange
authors.forename: C
authors.initials: C
authors.lastname: Segonds
authors.forename: X
authors.initials: X
authors.lastname: Troussard
authors.forename: G
authors.initials: G
authors.lastname: Flandrin
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D015463:Leukemia, Prolymphocytic / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D015461:Leukemia, Prolymphocytic, T-Cell / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate