Transient lupus anticoagulant associated with prothrombin deficiency: unusual cause of bleeding in a 5-year-old girl.

J E Humphries, M N Acker, J E Pinkston, S Ruddy
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Abstract

Purpose: We present the association of a lupus anticoagulant with hypoprothrombinemia in a 5-year-old girl, who presented with ecchymoses and a hematoma. This coagulopathy should be included in the differential of bleeding in the previously healthy children.

Patients and methods: Coagulation and immunology laboratory evaluation was performed at the time of presentation with bleeding and 2 months later, after complete clinical recovery.

Results: A 5-year-old girl presented with ecchymoses and a hematoma after after an upper respiratory illness. Laboratory evaluation showed prolongation of both the prothrombin time (PT) and activated partial thromboplastin time (aPTT) due to the presence of a strong lupus anticoagulant associated with a decreased level of prothrombin (15 U/dl). Hypocomplementemia was also detected. Bruising resolved spontaneously, and the PT and aPTT gradually normalized. Reevaluation 2 months later showed that the lupus anticoagulant had disappeared and the prothrombin deficiency was markedly improved.

Conclusions: This case demonstrates that transient lupus anticoagulants must be included in the differential for bleeding in young children. Also, in children with lupus anticoagulants, neither the association of hypoprothrombinemia nor the presence of evidence of activation of the immune system appears to predict whether a patient will have or develop systemic lupus erythematosus.

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短暂性狼疮抗凝血剂与凝血酶原缺乏:出血的不寻常原因在一个5岁的女孩。
目的:我们提出狼疮抗凝血剂与低凝血酶原血症的关联在一个5岁的女孩,谁提出了淤血和血肿。这种凝血功能障碍应包括在先前健康儿童出血的鉴别中。患者和方法:在出现出血时和临床完全恢复后2个月进行凝血和免疫学实验室评估。结果:一名5岁女孩在上呼吸道疾病后出现瘀斑和血肿。实验室评估显示凝血酶原时间(PT)和活化的部分凝血活酶时间(aPTT)的延长,由于存在与凝血酶原水平降低(15 U/dl)相关的强狼疮抗凝剂。还检测到补体不足。瘀伤自行消退,PT和aPTT逐渐恢复正常。2个月后复查显示狼疮抗凝剂消失,凝血酶原缺乏明显改善。结论:本病例表明,短暂性狼疮抗凝剂必须包括在鉴别出血的幼儿。此外,在使用狼疮抗凝剂的儿童中,无论是低凝血酶原血症的相关性,还是存在免疫系统激活的证据,似乎都不能预测患者是否会患有或发展为系统性红斑狼疮。
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