BCR/ABL-1-Positive Myeloproliferative Neoplasm Presenting with Isolated Remarkable Thrombocytosis with Atypical Clinicopathological Features: Discussion from Management Point of View

Abstract

Chronic Myeloid Leukaemia can rarely present in essential Thrombocythaemia-like picture. Apart from the genetic defining marker (BCR-ABL fusion), these cases lack almost all typical features of CML. Here, we highlight the response of these patients to different therapeutic approaches and to emphasize that although the proliferation is solely limited to the platelets; this group of patients did not show any response except after initiation of Tyrosine kinase inhibitors which highpoints the essentiality of excluding CML by performing BCR/ ABL-1 in all cases with features of myeloproliferative neoplasms in order to avoid delayed management and adverse outcome. Apparently, many hematologists have not been persuaded to always test for BCR/ABL-1 when there are no features suggesting CML. Unlike what was previously reported, upon literature review, we found no significant difference in disease prognosis in this group of patients compared to classic CML, provided TKI was started early in disease course.