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

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 p230BCR-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.