真性红细胞增多症继发颅内静脉窦血栓形成两例临床分析

Clinical Analysis of Cerebral Venous Sinus Thrombosis Secondary to Polycythemia Vera

目的探讨真性红细胞增多症(PV)继发脑静脉窦血栓形成的临床表现及血液学、影像学特点。方法回顾性分析2例确诊的PV患者继发脑静脉窦血栓形成的临床资料。结果2例患者均以头痛、恶心、呕吐为首发表现,伴有皮肤、黏膜改变及脾脏肿大,测脑脊液压力均升高,但常规生化检查均正常,血液形态学检查显示红细胞、白细胞、血小板三系增多,骨髓象检查显示细胞增生活跃,颅脑磁共振静脉成像检查显示受累静脉窦内条索状长T1长T2信号影,颅脑MRV显示受累的静脉窦不显影或显影不清。结论PV可以继发脑静脉窦血栓形成,常规颅脑MRI检查及行血液形态学、骨髓穿刺检查可以明确诊断。

Objective To study the clinical manifestations, hematological and imaging features of cerebral venous sinus thrombosis （CVST） secondary to polycythemia vera （PV）. Methods A retrospective analysis was performed on clinical data of 2 patients with CVST secondary to PV. Results Both patients had headache, nausea and vomiting as initial performances, accompanied by skin and mucous membrane changes, enlarged spleen, elevated cerebrospinal fluid pressure, but with normal conventional biochemical indexes. Blood morphological examination showed increased red blood cells, white, platelets; bone marrow picture showed active cellular proliferation; brain MRI examination revealed cord - like long T1, long T2 signal shadow in involved venous sinus; brain MRV displayed no or unclear visualization of involved venous sinus. Conclusion PV, which can lead to CVST, can be conformed by conventional brain MRI, blood morphology, marrow puncture.