A case report of Chronic myelogenous leukemia presenting as blastic crisis with a T-cell acute lymphoblastic leukemia phenotype: Awareness of a rare entity

Acta Haematol. 2023 Aug 9. doi: 10.1159/000529911. Online ahead of print.ABSTRACTChronic Myelogenous leukemia at blast crisis with a T-cell phenotype (T-ALL CML-BC ) at diagnosis, without any prior history of CML is extremely rare. After the introduction of TKIs, CML patients have a median survival comparable to general population and accelerated/blast crisis are rarely encountered. Most CML patients (80%) transform into AML and the rest into B-ALL. Anecdotal cases of Ph+ T-ALL, either de novo or in the context of CML BC have been reported .Left shift in the blood, the presence of splenomegaly/extramedullary infiltration and the occurrence of bcr-abl rearrangement in both the blastic population, as well as in the myeloid cell compartment are key points in differentiating de novo Ph+ T-ALL from T-ALL CML-BC. The latter is a rare entity, characterized by extramedullary disease , p210 transcript and clonal evolution. Lack of preceding CML does not rule out the diagnosis of T-ALL CML-BC. Prompt TKI treatment with ALL-directed therapy followed by allo-SCT may offer long-term survival in this otherwise poor prognosis entity. In this paper we describe a patient with T-ALL CML -BC at presentation, still alive 56 months after diagnosis and we offer a review of the literature on this rare subject .All clinical and laboratory features are provided in order to distinguish de novo Ph+ T-ALL from T-ALL CML-BC, underscoring the prognostic and therapeutic significance of such a differentiati...
Source: Acta Haematologica - Category: Hematology Authors: Source Type: research