Lucijanic Marko, Krecak Ivan, Soric Ena, Sabljic Anica, Galusic Davor, Holik Hrvoje, Perisa Vlatka, Moric Peric Martina, Zekanovic Ivan, Budimir Leonardo, Kusec Rajko
Hematology Department, University Hospital Dubrava, Av. Gojka Suska 6, 10000 Zagreb, Croatia.
Scientific Research and Translational Medicine Department, University Hospital Dubrava, Av. Gojka Suska 6, 10000 Zagreb, Croatia.
Cancers (Basel). 2025 Apr 22;17(9):1390. doi: 10.3390/cancers17091390.
: Overt primary myelofibrosis (PMF), secondary post-polycythemia vera (post-PV), and post-essential thrombocythemia (post-ET) myelofibrosis (SMF) are chronic myeloproliferative neoplasms (MPN) that sometimes present with extreme thrombocytosis (ExTh, platelet count > 1000 × 10/L), a phenomenon of uncertain clinical significance since there are no published data available. : We retrospectively investigated the clinical correlations and associated outcomes of ExTh in a cohort of 172 patients with overt myelofibrosis diagnosed in six Croatian hematology centers. : ExTh was present in 5.8% of patients and was associated with post-ET etiology of myelofibrosis, older age, smaller spleen size, and the presence of arterial hypertension ( < 0.05 for all analyses). No significant associations were observed with sex, degree of bone marrow fibrosis, or driver mutation status. Over the follow-up period, patients with ExTh experienced a favorable course regarding survival ( < 0.001) and bleeding risk ( = 0.034), whereas no significant association with thrombotic risk was observed ( = 0.682). : In contrast to its context in ET, ExTh in overt fibrotic MPN does not appear to confer higher bleeding or thrombotic risk. Instead, it is associated with more favorable survival outcomes and reduced bleeding risk.
原发性骨髓纤维化（PMF）、真性红细胞增多症后骨髓纤维化（post-PV）和原发性血小板增多症后骨髓纤维化（post-ET）（SMF）是慢性骨髓增殖性肿瘤（MPN），有时会出现极端血小板增多症（ExTh，血小板计数>1000×10⁹/L），由于尚无公开数据，这一现象的临床意义尚不确定。我们回顾性研究了在六个克罗地亚血液学中心诊断的172例原发性骨髓纤维化患者队列中ExTh的临床相关性及相关结局。5.8%的患者存在ExTh，其与骨髓纤维化的post-ET病因、老年、脾脏较小以及动脉高血压有关（所有分析P<0.05）。未观察到与性别、骨髓纤维化程度或驱动基因突变状态有显著关联。在随访期间，ExTh患者在生存方面（P<0.001）和出血风险方面（P=0.034）经历了较好的病程，而未观察到与血栓形成风险有显著关联（P=0.682）。与ET中的情况相反，原发性纤维化MPN中的ExTh似乎不会带来更高的出血或血栓形成风险。相反，它与更有利的生存结局和降低的出血风险相关。
