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Can p230 BCR-ABL myeloproliferative neoplasm be distinguished from chronic myelogenous leukaemia?


Hématologie. Volume 15, Number 6, 416-23, novembre-décembre 2009, Revue

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Author(s) : Caroline Mayeur-Rousse, Laureen Aumerle, Nathalie Sorel, Jean-Claude Chomel, Ali G Turhan

Summary : Neutrophilic Chronic Myeloid Leukaemia (N-CML) has been described as a Chronic Myelogenous Leukaemia (CML) variant in the early 90’s. This hematologic malignancy originates from a t(9\;22) translocation (presence of the Philadelphia chromosome) leading to a specific BCR-ABL e19a2 molecular rearrangement. The resulting Bcr-Abl oncoprotein of 230 kDa (p230 BCR-ABL) contains additional Bcr sequences including the first third of the Rac-GTPase domain, not found in p210 BCR-ABL protein, pathognomonic of CML. N-CML was characterized by a predominant peripheral blood neutrophilia and a more indolent course. Based on the few cases published in literature, we investigated the potential relationship between the presence of p230 BCR-ABL oncoprotein and a particular phenotype. Regarding their biologic properties, clinical and therapeutic features, we explore the question of whether CML and N-CML can be considered as two distincts entities.

Keywords : p230 BCR-ABL, chronic myelogenous leukaemia, myeloproliferative neoplasms, BCR-ABL

 

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