Raval Mihir, Paul Anu
Department of Internal Medicine, Sanford Hospital, Fargo, N. Dak., USA.
Case Rep Neurol. 2010 Nov 22;2(3):150-156. doi: 10.1159/000322571.
Cerebral venous thrombosis is a rare initial presentation of polycythemia. If diagnosed early, treatment can reduce mortality and morbidity significantly. Often it may present with headache as the only complaint, and thus the diagnosis is likely to be missed. CASE PRESENTATION: A medically stable 31-year-old male, a chronic smoker with a ∼17 pack-year history of smoking, was admitted to the emergency room with a 2-week history of gradually worsening, severe, throbbing headache in the occipital region sensitive to light. Initial neurological examination was positive only for some involuntary motor tics of the left leg. Initial laboratory workup showed hemoglobin of 20 g/dl and hematocrit of 56.5%. The carboxyhemoglobin level was normal, but the oxygen dissociation curve was shifted to the left. Further evaluation by MRI and MRA of the brain suggested extensive and complete thrombosis of the superior sagittal sinus, right transverse sinus and right sigmoid sinus with a small venous infarct in the right parafrontal region. Given that the patient first presented with a thrombotic event, workup for primary polycythemia and hypercoagulable disorders was carried out, including JAK2 mutation evaluation, which was negative. This left us with smoking as the only risk factor and possible cause for secondary polycythemia. He improved significantly with phlebotomy and anticoagulation treatment. CONCLUSION: This case illustrates a rare but severe complication of secondary polycythemia stressing the importance of being aware of the risk of developing cerebral thrombosis in patients with chronic smoking exposure.
脑静脉血栓形成是真性红细胞增多症罕见的首发表现。若早期诊断,治疗可显著降低死亡率和发病率。其常仅以头痛为唯一症状,故易漏诊。病例报告:一名31岁男性,病情稳定,有17年约1包年的慢性吸烟史,因枕部逐渐加重的严重搏动性头痛2周且对光敏感而入住急诊室。初始神经系统检查仅发现左腿有一些不自主运动抽搐。初始实验室检查显示血红蛋白为20 g/dl,血细胞比容为56.5%。碳氧血红蛋白水平正常,但氧解离曲线左移。脑部MRI和MRA进一步评估提示上矢状窦、右侧横窦和右侧乙状窦广泛且完全血栓形成,右侧额旁区域有小静脉梗死。鉴于患者首次出现血栓事件,对原发性真性红细胞增多症和高凝性疾病进行了检查,包括JAK2突变评估,结果为阴性。这使得吸烟成为继发性真性红细胞增多症的唯一危险因素和可能病因。经放血和抗凝治疗后,他病情明显改善。结论:本病例说明了继发性真性红细胞增多症一种罕见但严重的并发症,强调了认识到长期吸烟患者发生脑血栓风险的重要性。