Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville.
Service d'Hématologie Biologique, Hôpital Bretonneau.
Clin J Am Soc Nephrol. 2019 Apr 5;14(4):557-566. doi: 10.2215/CJN.11470918. Epub 2019 Mar 12.
Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a retrospective study in a four-hospital institution in 564 consecutive patients with adjudicated thrombotic microangiopathies during the 2009-2016 period. We estimated the incidence of primary and secondary thrombotic microangiopathies, thrombotic microangiopathy causes, and major outcomes during hospitalization (death, dialysis, major cardiovascular events [acute coronary syndrome and/or acute heart failure], and neurologic complications [stroke, cognitive impairment, or epilepsy]).
We identified primary thrombotic microangiopathies in 33 of 564 patients (6%; thrombotic thrombocytopenic purpura: 18 of 564 [3%]; atypical hemolytic and uremic syndrome: 18 of 564 [3%]). Secondary thrombotic microangiopathies were found in 531 of 564 patients (94%). A cause was identified in 500 of 564 (94%): pregnancy (35%; 11 of 1000 pregnancies), malignancies (19%), infections (33%), drugs (26%), transplantations (17%), autoimmune diseases (9%), shiga toxin due to (6%), and malignant hypertension (4%). In the 31 of 531 patients (6%) with other secondary thrombotic microangiopathies, 23% of patients had sickle cell disease, 10% had glucose-6-phosphate dehydrogenase deficiency, and 44% had folate deficiency. Multiple causes of thrombotic microangiopathies were more frequent in secondary than primary thrombotic microangiopathies (57% versus 19%; <0.001), and they were mostly infections, drugs, transplantation, and malignancies. Significant differences in clinical and biologic differences were observed among thrombotic microangiopathy causes. During the hospitalization, 84 of 564 patients (15%) were treated with dialysis, 64 of 564 patients (11%) experienced major cardiovascular events, and 25 of 564 patients (4%) had neurologic complications; 58 of 564 patients (10%) died, but the rates of complications and death varied widely by the cause of thrombotic microangiopathies.
Secondary thrombotic microangiopathies represent the majority of thrombotic microangiopathies. Multiple thrombotic microangiopathies causes are present in one half of secondary thrombotic microangiopathies. The risks of dialysis, neurologic and cardiac complications, and death vary by the cause of thrombotic microangiopathies.
血栓性微血管病构成了诊断和治疗方面的挑战。继发性血栓性微血管病不如原发性血栓性微血管病（血栓性血小板减少性紫癜和非典型溶血尿毒综合征）那么有特征性。继发性和原发性血栓性微血管病的相对频率和结局尚不清楚。
方法、设置、参与者和测量：我们对 2009 年至 2016 年期间在 4 家医院机构的 564 例经裁决的血栓性微血管病患者进行了回顾性研究。我们估计了原发性和继发性血栓性微血管病的发生率、血栓性微血管病的病因以及住院期间的主要结局（死亡、透析、主要心血管事件[急性冠状动脉综合征和/或急性心力衰竭]和神经并发症[中风、认知障碍或癫痫]）。
我们在 564 例患者中发现了 33 例原发性血栓性微血管病（6%；血栓性血小板减少性紫癜：564 例中的 18 例[3%]；非典型溶血尿毒综合征：564 例中的 18 例[3%]）。564 例中有 531 例（94%）为继发性血栓性微血管病。在 564 例中有 500 例（94%）确定了病因：妊娠（35%；1000 例妊娠中有 11 例）、恶性肿瘤（19%）、感染（33%）、药物（26%）、移植（17%）、自身免疫性疾病（9%）、志贺毒素（6%）和恶性高血压（4%）。在 531 例（6%）有其他继发性血栓性微血管病的患者中，23%的患者患有镰状细胞病，10%的患者患有葡萄糖-6-磷酸脱氢酶缺乏症，44%的患者患有叶酸缺乏症。继发性血栓性微血管病比原发性血栓性微血管病更常出现多种血栓性微血管病病因（57%比 19%；<0.001），且主要是感染、药物、移植和恶性肿瘤。血栓性微血管病病因之间存在显著的临床和生物学差异。在住院期间，564 例患者中有 84 例（15%）接受了透析治疗，564 例患者中有 64 例（11%）发生了主要心血管事件，564 例患者中有 25 例（4%）发生了神经并发症；564 例患者中有 58 例（10%）死亡，但并发症和死亡的发生率因血栓性微血管病的病因而异。
继发性血栓性微血管病占血栓性微血管病的大多数。一半的继发性血栓性微血管病存在多种血栓性微血管病病因。透析、神经和心脏并发症以及死亡的风险因血栓性微血管病的病因而异。
