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Multiple Lacunar Infarcts in Paroxysmal Nocturnal Hemoglobinuria

机译:在阵发性夜间血红蛋白尿的多个凝结性梗塞

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摘要

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease characterized by clonal hematopoietic stem cell disorder, with increased mortality and morbidity. Venous thrombosis is the most common cause of mortality in PNH. The relationship between PNH and cerebrovascular disease is unclear; few cases are reported in the literature, most of them related to cerebral venous thrombosis; In PNH the involvement of intracranial and extracranial arterial sites is very rare. We report a case of a 49-year-old woman who has a medical history of diabetes mellitus, hypertension, and PNH and presented multiple lacunar strokes in a routine consultation with a hematologist. A brain computed tomography (CT) scan showed lacunar infarcts, and magnetic resonance image showed acute ischemic stroke, multiple territory lacunar infarctions, and focal area of microbleeds in gradient echo sequence. A CT angiography showed V3 and V4 branches of the left vertebral artery occluded by a thrombus, and the posterior inferior cerebellar artery occluded, whereas the carotid system was normal. We discuss the presentation and physiopathology of stroke in PNH and other cases reported in the literature review. ]]>
机译:阵发性夜间血红蛋白尿(PNH)是一种罕见的患病,其特征在于克隆造血干细胞疾病,具有增加的死亡率和发病率。静脉血栓形成是PNH中最常见的死亡原因。 PNH和脑血管疾病之间的关系尚不清楚;在文献中报道了很少的病例,其中大多数与脑静脉血栓形成有关;在PNH中,颅内和颅内动脉位点的参与是非常罕见的。我们举报了一个49岁女性的案例,糖尿病病史,高血压和PNH,并在与血液学学者的常规咨询中呈现多个凝聚程度。脑计算断层扫描(CT)扫描显示出LELUNAR梗死,磁共振图像显示急性缺血性卒中,多个境内狭窄梗塞和微微型回波序列的焦斑术焦点。 CT血管造影显示由血栓闭塞的左椎动脉的V3和V4分支,并且后部下小脑动脉堵塞,而颈动脉系统是正常的。我们讨论了在文献综述中报告的PNH和其他病例中卒中的展示和生理病理学。 ]]>

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