摘要
目的探讨低凝血酶原血症-狼疮抗凝物综合征(HLAS)的病因、临床表现、诊断和治疗。方法回顾性分析我院确诊的1例伴出血时间延长的HLAS患者的临床特点,并结合文献报道加以归纳总结。结果HLAS临床表现差异很大,与体内凝血酶原活性不完全平行。年轻患者病因以结缔组织病和病毒感染为主,前者激素治疗通常有效,少数需要加用免疫抑制剂或静脉注射用免疫球蛋白(IVIG);后者主要发生在儿童,通常在3个月内自发缓解,个别患者需要使用激素治疗。年长者常见病因为肿瘤、药物相关或原因不明。结论对于不明原因的出血患者,应警惕HLAS的可能。
Objective To explore the etiology, diagnosis and treatment of hypoprothrombinemia-lupus anticoagulant syndrome （HLAS）. Methods The clinical characteristics and laboratory results of 1 case of HLAS with prolonged bleeding time was analyzed, and the literature was reviewed. Results The clinical manifestation of HLAS varies, from asymptomatic to severe hemorrhage. HLAS was strongly associated with autoimmune diseases in young adults, and glucocorticoids were effective in majority, immunosuppressor or intravenous immunoglobulin （WIG） was also needed in few cases. Virus associated HLAS was mostly found in children, and can be self-limited, and spontaneous remission within 3 months, few needed glucocoricoids. The main causes of HLAS in adults were tumor, drug related or unknown. Conclusion HLAS is rare. Cases of HLAS should be thoroughly investigated for any known causes and related disorders.
出处
《血栓与止血学》
2010年第2期82-85,91,共5页
Chinese Journal of Thrombosis and Hemostasis
