Indolent course as a relatively frequent presentation in T‐prolymphocytic leukaemia

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.

High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia

“…This apparent TCR and TCL1 cooperativity may also be related to the previously identified association of outcome in T-PLL with CD45RA versus CD45RO expression or to coexpression of the CD4 and CD8 coreceptors. 8,13,39 The ability of TCL1 to modulate AKT signaling through a variety of stimuli also has important implications for its role in lymphocyte biology. 40 Dysregulated TCL1 as a result of specific chromosomal aberrations has not been shown in other lymphatic tumors.…”

“…6,[10][11][12] Although there are few large series, several immunophenotypic tumor subsets with differing outcomes likely exist. 6,8,[13][14][15][16][17] TCL1, which is not expressed in nonneoplastic mature T cells, is one member of a family of small ␤-barrel proteins found predominantly in the cytoplasm whose mechanism of action remains unresolved. Rare cases of T-PLL overexpress another TCL1 homolog, MTCP1, through a X;14 translocation.…”

High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H

“…1 Although some patients may initially present with indolent disease, they eventually progress and the outcome is then similar. 2 New therapeutic approaches are badly needed to improve outcome in this fatal disease.…”

A Rare Presentation of Cutaneous Pseudolymphoma as a Preleukaemic State

“…The characteristic clinical features of TPLL include splenomegaly, lymphadenopathy, skin disease and a high leukocyte count. The cutaneous features occurred in 25-28% of cases of TPLL in various case series [7,8]. Skin disease has been reported at the time of initial diagnosis or later, heralding aggressive clinical course in such cases.…”