原发性血小板增多症合并脑静脉窦血栓形成患者的临床分析

Clinical characteristics of essential thrombocythemia combined with cerebral venous sinus thrombosis

目的探讨原发性血小板增多症(ET)合并脑静脉窦血栓形成(CVST)的临床特点及预后,以提高诊疗水平。方法回顾性分析2016年5月至2019年6月于首都医科大学宣武医院连续住院治疗的14例ET合并CVST患者的临床资料,同时按1∶2的比例纳入同一时期在本院住院治疗的28例非CVST的ET患者作为对照组。收集所有患者的一般资料、发病特点、神经系统症状和体征、脑脊液检查、实验室检验、影像学检查、治疗及预后等临床资料,应用原发性血小板增多症血栓国际预后评分(IPSET)对患者进行血栓风险分层,对两组患者的临床特点、相关危险因素及治疗情况进行对比。结果共纳入ET合并CVST患者14例,其中男6例,女8例;年龄17~55岁,中位年龄为29.5(24.5,44.0)岁;病程中位数为150(20,565)d。慢性起病8例,亚急性起病5例,急性起病1例。CVST的主要首发症状为头痛。临床表现主要包括头痛(13/14)、恶心呕吐(8/14)及视物模糊(7/14),其中8例合并视神经乳头水肿。影像学检查显示静脉性脑梗死6例,其中4例合并出血转化。所有患者表现为2处及以上静脉窦受累,常见的血栓部位分别为横窦(12/14)、乙状窦(12/14)、上矢状窦(9/14)及颈内静脉(7/14)。14例患者接受抗凝治疗,6例接受降细胞治疗。13例患者预后良好,无复发及死亡。ET合并CVST组的年龄[29.5(24.5,44.0)岁比47.0(38.8,54.8)岁,P=0.003]、血小板计数[(518±110)×10^9/L比(777±253)×10^9/L,P<0.01]明显低于未合并CVST的对照组。结论CVST是ET的少见并发症。对于无明确诱因的CVST伴血小板计数增多患者,需考虑ET的可能;ET患者出现颅高压症状时,应尽早完善CVST的相关检查。早诊断、规范抗栓及降细胞治疗可改善ET合并CVST患者的预后。

Objective To explore the clinical characteristics and prognosis of essential thrombocythemia(ET)complicated with cerebral venous sinus thrombosis(CVST).Methods A total of fourteen patients who were diagnosed as ET complicated with CVST at Xuanwu Hospital,Capital Medical University from May 2016 to June 2019,were enrolled and analyzed retrospectively.Simultaneously,ET patients without CVST(at 1∶2 ratio)diagnosed in the same period were selected as control.The data of demographic characteristics,clinical course,neurologic symptoms and signs,the result of cerebrospinal fluid,imaging characteristics,laboratory examinations,outcomes,and prognosis of all patients were collected and analyzed.The clinical characteristics,associated risk factors,and treatment outcomes were analyzed between two groups.The International Prognostic Score of thrombosis for essential thrombocythemia(IPSET)was used to stratify the risk of thrombosis in patients.Results Fourteen ET patients with CVST were enrolled,including 8 females and 6 males.The median age and disease duration at the onset of CVST were 29.5(24.5,44.0)years old and 150(20,565)days,respectively.Most forms of the clinical course were chronic(8/14),followed by subacute(5/14)and acute(1/14).The mainly first symptom of CVST was headache(13/14),followed by nausea/vomiting(8/14)and visual loss(7/14).Eight of 14 patients were detected with papilledema.Imaging examination showed 6 cases of venous cerebal infarction,of which 4 cases were complicated with hemorrhagic transformation.Multiple sinuses thrombosis were involved in all patients with common locations of CVST in the transverse sinus(12/14),sigmoid sinus(12/14),superior sagittal sinus(9/14),and internal jugular vein(7/14).Among ET patients with CVST,14 cases were treated with anticoagulants,and 6 cases were treated with cytoreductive therapy.Thirteen of 14 patients achieved favorable prognosis,and there was no recurrence and death during follow-up.Compared to ET patients without CVST,ET patients with CVST had significantly smaller ages(29.5[24.5,44.0]vs.47.0[38.8,54.8],P=0.003)and lower platelet counts([518±110]×10^9/L vs.[777±253]×10^9/L,P<0.01).Conclusions CVST is a rare complication of ET.The possibility of ET should be considered in unexplained CVST patients with thrombocythemia.Evaluation of CVST should be performed for ET patients with headache,nausea/vomiting,and papilledema.Early diagnosis,standard anticoagulation,and cytoreductive therapy can improve the prognosis of ET patients with CVST and prevent a recurrence.