Hasui Hidenari, Iseki Tatou, Ueno Yuji, Kamiyama Daiki, Miyamoto Nobukazu, Kijima Chikage, Hira Kenichiro, Komatsu Norio, Hattori Nobutaka
Department of Neurology, Juntendo University Faculty of Medicine, Tokyo, Japan.
Department of Hematology, Juntendo University Faculty of Medicine, Tokyo, Japan.
Case Rep Neurol. 2022 Jun 7;14(2):274-280. doi: 10.1159/000525171. eCollection 2022 May-Aug.
Polycythemia vera (PV) is one of the myeloproliferative neoplasms and has higher frequency of the V617F mutation. Hemorrhagic stroke is rare in PV, and myelofibrosis is secondary to PV. A 76-year-old Japanese man was diagnosed as PV with the V617F mutation at the age of 63 years. He developed anemia together with secondary myelofibrosis, and then 40 mg ruxolitinib was started at 70 years. At 76 years, he presented with apathy and was diagnosed with intracerebral hemorrhage (ICH) in the right thalamus. Six months later, he developed multiple ICHs in bilateral cerebellar hemispheres. Leukocyte count was 57,600/μL, platelet count was 66,000/μL, and the level of hemoglobin was 8.7 g/dL. Bleeding time was 6 min. The agglutination ability when adding collagen was 41%. A patient with the V617F mutation developing hemorrhagic stroke due to late-stage PV and secondary myelofibrosis was reported, implying various mechanisms for recurrent and multiple ICH.
真性红细胞增多症（PV）是一种骨髓增殖性肿瘤，V617F突变的发生率较高。PV患者发生出血性卒中较为罕见，骨髓纤维化是PV的继发性病变。一名76岁的日本男性在63岁时被诊断为携带V617F突变的PV。他出现了贫血并伴有继发性骨髓纤维化，70岁时开始使用40mg芦可替尼治疗。76岁时，他出现淡漠症状，被诊断为右侧丘脑脑出血（ICH）。6个月后，他双侧小脑半球出现多发ICH。白细胞计数为57,600/μL，血小板计数为66,000/μL，血红蛋白水平为8.7g/dL。出血时间为6分钟。添加胶原蛋白时的凝集能力为41%。本文报道了一名携带V617F突变的患者因晚期PV和继发性骨髓纤维化发生出血性卒中，提示复发性和多发性ICH存在多种机制。
