災難性抗磷脂症候群

Catastrophic Antiphospholipid Syndrome

楊登和(Deng-Ho Yang) ； 陳政宏(Chen-Hung Chen)

《內科學誌》 20卷4期 (2009/08) Pp. 335-343

https://doi.org/10.6314/JIMT.2009.20(4).07

摘要

災難性抗磷脂症候群(catastrophic antiphospholipid syndrome)爲一少見且影響多處器官形成栓塞之自體免疫性疾病，在所有抗磷脂症候群病人中所佔比例少於1%，不過通常爲致命性的情況，所以在臨床上要有很高的警覺性；很多誘發因子都有可能造成災難性抗磷脂症候群的出現，包括：感染、外傷或手術、癌症、狼瘡發作、產科疾病、Warfarin不足、藥物等；儘管在抗凝血劑的治療下，這多重器官衰竭的疾病，其死亡率還是高達50%。早期診斷與適時給予抗凝血劑、類固醇、血漿置換術或靜脈注射免疫球蛋白治療對病人疾病的控制與死亡率的下降是相當重要的。

關鍵字

災難性抗磷脂症候群 ； 血栓 ； 多重器官衰竭 ； 血栓風暴

並列摘要

Catastrophic antiphospholipid syndrome is an uncommon autoimmune disorder with multiple organ involvement due to a widespread thrombotic process. The patients with catastrophic antiphospholipid syndrome represent less than 1% of all patients with antiphospholipid syndrome, and they are usually in a life-threatening medical condition that requires high clinical awareness. The direct precipitating factors induced catastrophic antiphospholipid syndrome include infection, trauma or surgical procedures, neoplasia, lupus flares, obstetric complications, wafarin withdrawal and drug administration. In this multiple organ failure associated disorder, the mortality rate is around 50% with the use of anticoagulation therapy. Early diagnosis and adequate treatment by anticoagulation, steroid, plasma exchange or intravenous immunoglobulins is important for control of the disease's activity and decreased the mortality rate in this disorder.

並列關鍵字

Catastrophic antiphospholipid syndrome ； Thrombosis ； Multiple organ failure ； Thrombotic storm