Analysis of clinical characteristics and efficacy of chronic myeloid leukemia onset with extreme thrombocytosis in the era of tyrosine kinase inhibitors

Abstract

The aim of this study was to investigate the clinical characteristics and efficacy of chronic myeloid leukemia (CML) onset with extreme thrombocytosis. A total of 121 newly diagnosed and untreated CML patients in chronic phase with complete clinical information from the First Hospital of Jilin University, from January 2010 to December 2014 were retrospectively recruited. Based on the platelet (PLT) count, 22 patients were assigned into CML with thrombocytosis (CML-T) group (PLT >1,000×109/L) and 65 patients were classified into CML without extreme thrombocytosis (CML-N) group (PLT ≤1,000×109/L). Fifty-four point five percent of patients in the CML-T group were female, which was higher than that in the CML-N group (27.7%) (P=0.022). Except for gender, there was no significant difference for clinical information of patients between the two groups. For Sokal and Hasford scoring systems, the percentage of patients at high risk in the CML-T group were higher than those in the CML-N group, 95.5% vs 52.3% (P=0.000) and 68.2% vs 41.5% (P=0.031), respectively; however, there was no significant difference for European Treatment and Outcome Study (EUTOS) score system between the two groups (P=0.213). In terms of major molecular response (MMR) rate, the percent of patients with MMR in CML-T group was lower than that in CML-N group at 36 months after tyrosine kinase inhibitor therapy (P=0.037). Up until December 2016, the median of event-free survival was 21 months in the CML-T group, however, that was not reached in the CML-N group (P=0.027). The majority of CML patients with extreme thrombocytosis were females, and compared to patients in the CML-N group, the percentage of high risk patients based on the Sokal and Hasford scoring systems was higher in the CML-T group, and the median event-free survival of patients was shorter.

Chronic Myeloid Leukemia: Atypical Presentation and Diagnostic Pitfall in the Workup of Isolated Thrombocytosis

“…Patients with CML are, in many cases, asymptomatic at diagnosis and only incidentally detected upon abnormalities found on routine blood testing. CML is the most common condition in the spectrum of MPDs [3]. It typically presents with leukocytosis, with elevated immature granulocytes, basophilia, and eosinophilia in the PB [3,4].…”

“…CML is the most common condition in the spectrum of MPDs [3]. It typically presents with leukocytosis, with elevated immature granulocytes, basophilia, and eosinophilia in the PB [3,4].…”

“…Thrombocytosis, described as a platelet count of more than 450×10 9 /L, is common in chronic MPDs [3]. Thrombocytosis could be reactive or it may be paraneoplastic where it could be triggered by altered immune response to MPDs, including polycythemia vera, CML, or ET [4,6].…”

Bleeding signs due to acquired von Willebrand syndrome at diagnosis of chronic myeloid leukaemia in children

“…It is generally reported that thrombocytosis in adult CML patients has no close relationship with haemorrhagic and thrombotic events. When single centre studies investigated the episodes of haemorrhagic and thrombotic events, 22 out of 85 (26%) adult Chinese CML patients were assigned into CML with thrombocytosis but only one patient was detected with thrombosis at diagnosis, and no case with bleeding was observed (Liu et al , ). In an analysis from a single Italian centre, only one out of 100 adult CML patients developed thrombosis at diagnosis, and no history of bleeding was reported (Sorà et al , ).…”

“…This proportion observed is in the range of haemostatic complications (10Á3%) reported for adults with CML at diagnosis. But it must be stressed that the data for adult cohorts usually comprise the sum of thrombotic and haemorrhagic complications (Schafer, 1984;Liu et al, 2017;Sora et al, 2018) while no case of thrombosis was observed in childhood CML although the proportion of patients exhibiting a platelet count >1 000 9 10 9 /l was considerably higher. Besides CML, Philadelphia chromosome-negative myeloproliferative neoplasms (MPN, polycythaemia vera [PV], essential thrombocythaemia [ET], primary myelofibrosis [MF]) are associated with thrombotic events occurring frequently at unusual sites (portal and splenic veins, mesenteric arteries, cerebral sinuses).…”