Clinical analysis of 8 cases of connective tissue disease complicated with acquired hemophilia A
10.3760/cma.j.issn.1007-7480.2019.04.008
- VernacularTitle:结缔组织病合并获得性血友病甲八例临床分析
- Author:
Yanli YANG
1
;
Pengyan QIAO
;
Yu CHEN
;
Ke XU
;
Gailian ZHANG
;
Liyun ZHANG
Author Information
1. 山西医学科学院山西大医院风湿免疫科
- Keywords:
Connective tissue diseases;
Lupus erythematosus;
systemic;
Sj(o)gren's syndrome;
Arthritis rheumatoid;
Acquired hemophilia A;
Activated partial thromboplastin time prolongation;
Rituximab
- From:
Chinese Journal of Rheumatology
2019;23(4):259-262
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical features and treatment of connective tissue disease (CTD) complicated with acquired hemophilia A (AHA).Methods A retrospective analysis of 8 cases of CTD [5 cases of systemic lupus erythematosus (SLE),2 cases of Sj(o)gren's syndrome (SS),1 case of rheumatoid arthritis (RA)] related to clinical manifestations,diagnostic methods,treatment options and outcomes.Results At the onset of AHA,active disease was shown in 7 patients with CTD,and 5 cases had bleeding symptoms in different parts.There were 3 cases of anti-phospholipid syndrome in 5 cases of SLE,2 of which had thrombosis.In 8 patients,the activated partial thromboplastin time (APTF) was prolonged by 1.7 to 3.times,FⅧ∶ C was 9.2％ to 21％ (50％ to 150％),and the factor Ⅷ inhibitor titer was increased by 7.6 to 56 BU/m1 (Bethesda method).Seven patients were treated with sufficient hormones,immunosuppressive agents,human immunoglobulin (IVIG),and blood products.Five patients had clinically improved bleeding tendency and APIT,and one patient was ineffective.Conclusion CTD is easy to combine with AHA.Glucocorticoid combined with immunosuppressive agent can effectively treat CTD-related AHA.For refractory patients,rituximab can be an alternative.
