Department of Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal.
Department of Imunohemotherapy, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal.
J Thromb Thrombolysis. 2020 Feb;49(2):334-336. doi: 10.1007/s11239-019-01955-1.
Association of acquired factor II deficiency and lupus anticoagulant is a rare disease that can be related to sudden, severe or fatal haemorrhage. We present a 74-years-old woman with history of myelodysplastic syndrome, admitted to the Emergency Department due to spontaneous mucocutaneous bleeding. Coagulation assays revealed prolonged prothrombin time and activated partial thromboplastin time with evidence of an immediate acting inhibitor. Antithrombotic therapy usage, drug ingestion, disseminated intravascular coagulation, liver dysfunction and sepsis were excluded. Patient was admitted for close monitoring and etiological evaluation. A comprehensive bleeding diathesis workup was performed showing factor II levels severely decreased and transient positive lupus anticoagulant. Immunosuppression with methylprednisolone lasted for 3 days, followed by prednisolone. After 20 days she was discharged and follow-up was scheduled. Early diagnosis of lupus anticoagulant hypoprothrombinemia syndrome is critical, as it may result in fatal complications if not treated appropriately. There is no consensus regarding the best treatment, most being based on immunosuppression.
获得性凝血因子 II 缺乏症和狼疮抗凝物相关疾病较为罕见，可能与突发、严重或致命性出血相关。我们报告了一例 74 岁女性病例，该患者患有骨髓增生异常综合征，因自发性黏膜皮肤出血而被收入急诊。凝血检测显示凝血酶原时间和激活部分凝血活酶时间延长，存在即刻作用抑制剂。已排除抗凝治疗、药物摄入、弥漫性血管内凝血、肝功能障碍和脓毒症。患者被收住院密切监测并进行病因评估。进行了全面的出血倾向检查，结果显示凝血因子 II 水平严重降低且狼疮抗凝物呈一过性阳性。患者接受了 3 天的甲基强的松龙免疫抑制治疗，随后改为泼尼松龙。20 天后，患者出院并安排了随访。早期诊断狼疮抗凝物致低凝血酶原血症综合征至关重要，如果治疗不当，可能会导致致命并发症。针对该疾病的最佳治疗方法尚无共识，大多基于免疫抑制治疗。
