系统性红斑狼疮继发获得性血友病A 1例并文献复习

A case of acquired hemophilia A secondary to systemic lupus erythematosus and literature review

系统性红斑狼疮(SLE)是累及多器官、多系统的自身免疫性疾病,活动期常可累及血液系统,但导致凝血因子功能异常而继发获得性血友病A(AHA)相对罕见,易被误诊或漏诊。本文报道1例SLE继发AHA的青年女性患者,以头痛起病,活化部分凝血活酶时间明显延长,凝血因子Ⅷ活性明显下降,并逐渐出现面部蝶形红斑,血常规三系减少,最终诊断为SLE继发AHA。予糖皮质激素及免疫抑制剂治疗后病情缓解,凝血因子Ⅷ活性恢复正常。AHA主要临床表现多为近期自发性的急性出血,甚至危及生命。本文复习相关文献并进行总结,以期提高风湿科免疫科医生对AHA的认识。

Systemic lupus erythematosus(SLE)is an autoimmune disease that affects multiple organs and systems.During the active phase,it often affects the blood system,but leads to abnormal coagulation factor function and secondary acquired hemophilia A(AHA),which is relatively rare and easily misdiagnosed or missed.This article reports a young female patient with SLE secondary AHA who presented with headache onset,significant prolongation of activated partial thromboplastin time,significant decrease in coagulation factorⅧactivity,and gradual appearance of butterfly shaped erythema on the face.The blood routine showed a decrease in three series,and the final diagnosis was SLE secondary AHA.After treatment with glucocorticoids and immunosuppressants,the condition improved and the activity of coagulation factorⅧreturned to normal.The main clinical manifestations of AHA are recent spontaneous acute bleeding,even life-threatening.This article reviews relevant literature and summarizes it in order to enhance the understanding of AHA among immunologists in the department of rheumatism.