Can p230 BCR-ABL myeloproliferative neoplasm be distinguished from chronic myelogenous leukaemia? Volume 15, issue 6, novembre-décembre 2009


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Service d’hématologie et oncologie biologique, CHU de Poitiers ; EA 3805, Université de Poitiers, Poitiers

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.