中华临床免疫和变态反应杂志2018，Vol.12Issue(1)：19-24,6.DOI:10.3969/j.issn.1673-8705.2018.01.004
系统性红斑狼疮并发获得性血友病的临床特征
Clinical features of seven cases of systemic lupus erythematous associated with acquired hemophilia
摘要
Abstract
Objective To investigate the clinical features,treatment,and prognosis of systemic lupus erythematosus (SLE) with acquired hemophilia (AH).Methods Clinical data of 7 SLE patients with AH from January,2000 to August,2017 were retrospectively analyzed.The clinical manifestations and treatment were summarized.Results In the aspect of clinical subtypes of AH,one patient was diagnosed as Ⅻ factor deficiency,two patients were acquired vascular hemophilia (avWD) and the other four acquired hemophilia (AHA).Among the 7 patients,one had bulbar conjunctival hemorrhage,one had anal bleeding,and five presented extensive skin ecchymosis.Six patients had 1.2-to 7-fold prolonged activated partial thromboplastin time (APTT),while four patients had reduced factor Ⅷ (FⅧ) activity (0.1％-1％),and the level of FⅧ inhibitor increased to 32 to 972.8 Bethesda units/ml.There was a decreased level of vWF in 2 patients (1.6％-2.6％),and a decreased Ⅻ level in 1 patient (43.5％) as well.Transfusion of blood products followed by administration of glucocorticosteroids plus immunosuppressive agents,and intravenous immunoglobulin in six patients successfully arrested bleeding and improved APTT levels in 3 patients of AHA and 2 with avWD.Only one patient with AHA had no response to the above treatments.Conclusions SLE complicated with AH should be recognized and treated aggressively.Glucocorticosteroids combined with immunosuppressive could make a better prognosis for SLE with AH.关键词
红斑狼疮,系统性/获得性血友病/获得性血管性血友病/Ⅻ减少症Key words
Lupus erythematosus, systemic/Acquired hemophilia/Acquired von willebrand disease/Ⅻ factor deficiency引用本文复制引用
朱丽秀,曹晓宇,赵岩,曾小峰,张奉春,侯勇..系统性红斑狼疮并发获得性血友病的临床特征[J].中华临床免疫和变态反应杂志,2018,12(1):19-24,6.基金项目
北京协和医学院小规模特色办学经费支持项目(2015zlgc0110,2015zlgc0709)、中国医学科学院医学与健康科技创新工程项目(2017-12M-3-001)Small Scale Characteristic School Funds Support Project of Peking Union Medical College (2015zlgc0110,2015zlgc0709) （2015zlgc0110,2015zlgc0709）
Medical & Health Science Innovation Project of Chinese Academy of Medical Sciences (2017-12M-3-001) （2017-12M-3-001）
