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一名患有甲型流感相关血栓性血小板减少性紫癜的35岁女性。

A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura.

作者信息

Jonsson Maria K, Hammenfors Daniel, Oppegaard Oddvar, Bruserud Øystein, Kittang Astrid Olsnes

机构信息

aDepartment of Rheumatology bDivision for Infectious Diseases, Department of Medicine cDivision for Hematology, Department of Medicine, Haukeland University Hospital, Bergen dInstitute of Medicine, University of Bergen, Bergen, Norway.

出版信息

Blood Coagul Fibrinolysis. 2015 Jun;26(4):469-72. doi: 10.1097/MBC.0000000000000255.

Abstract

A previously healthy 35-year-old woman presented with severe thrombotic thrombocytopenic purpura (TTP) affecting several organs and concomitant influenza A infection. On admission to hospital, haemoglobin was 5.4 g/dl, platelet count 6 × 10/l, Schistocyte count in peripheral blood 5%, and throat swab positive for influenza A RNA. The patient was treated with antiviral medication and transfusions of fresh frozen plasma before plasma exchange therapy with excellent response. Plasmaphereses were attenuated after 5 days, resulting in TTP relapse 3 days later. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) protein was very low (<0.04 U/l) and anti-ADAMTS13 elevated (>2 U/l), consistent with acquired TTP. Platelet counts normalized after five additional plasma exchanges and oral corticosteroids. Antinuclear antibodies and subgroup anti-Ro/Sjögren's syndrome A antigen (SSA) were detected in serum and have remained borderline-elevated, although evaluation during TTP, recovery and follow-up have lacked clinical manifestations of connective tissue disease. Influenza A infection induced production of ADAMTS13 inhibitor, which resulted in TTP in a patient with circulating antinuclear antibodies, lacking other manifestations of connective tissue disease.

摘要

一名既往健康的35岁女性出现了累及多个器官的严重血栓性血小板减少性紫癜(TTP)并伴有甲型流感感染。入院时,血红蛋白为5.4g/dl,血小板计数为6×10⁹/l,外周血裂体细胞计数为5%,咽拭子甲型流感RNA检测呈阳性。患者在进行血浆置换治疗前接受了抗病毒药物治疗和新鲜冰冻血浆输注,反应良好。血浆置换在5天后减量,3天后导致TTP复发。具有血小板反应蛋白1型基序的解聚素和金属蛋白酶13(ADAMTS13)蛋白水平极低(<0.04U/l),抗ADAMTS13水平升高(>2U/l),符合获得性TTP。在额外进行了5次血浆置换和口服皮质类固醇治疗后,血小板计数恢复正常。血清中检测到抗核抗体和抗Ro/干燥综合征A抗原(SSA)亚组,尽管在TTP、恢复和随访期间的评估均未发现结缔组织病的临床表现,但这些指标仍处于临界升高状态。甲型流感感染诱导了ADAMTS13抑制剂的产生,导致一名循环抗核抗体阳性、无其他结缔组织病表现的患者发生TTP。

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