儿童狼疮抗凝血-低凝血原血症综合征1例报告。

IF 1
Wenling Shang, Xia Zhang, Rui Shi, Lei Ye, Yuefang Wang, Luyun Peng
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摘要

狼疮抗凝血-凝血酶低原血症综合征(LAHPS)是一种以狼疮抗凝血药物阳性和凝血酶原(血浆凝血因子II)降低为特征的疾病。LAHPS的主要症状是不同程度的出血。LAHPS是罕见的,目前没有明确的管理建议。部分本病患者临床表现不典型,易与其他疾病混淆,造成误诊。方法:我们报告一例lahyps在男性儿童谁最初表现为复发性鼻出血和过去的历史瘀斑。凝血酶原和活化的部分凝血活酶时间延长,血浆凝血因子II活性降低,狼疮抗凝剂阳性。随后出现继发性眼球出血、腰痛、发热等症状。结果:最终,根据他的自身抗体结果和其他测试,诊断为SLE。给予甲基强的松龙后,凝血逐渐改善,未见出血。讨论:在本报告中,血浆混合研究显示了抑制和辅助因子的作用,为该患者的疾病后续诊断和治疗提供了基础。本病例重点分析了实验室检测结果,对临床诊断和治疗有参考价值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lupus anticoagulant-hypoprothrombinemia syndrome in a child: a case report.

Introduction: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a disease characterized by positive lupus anticoagulant and decreased prothrombin (plasma coagulation factor II). The primary LAHPS symptom is varying degrees of bleeding. LAHPS is rare, and at present there are no clear recommendations for its management. Some patients with this disease have atypical clinical manifestations that are easily confused with other diseases, resulting in misdiagnosis.

Methods: We report a case of LAHPS in a male child who initially presented with recurrent epistaxis and past history of ecchymosis. Prothrombin and activated partial thromboplastin times were prolonged, plasma coagulation factor II activity was decreased, and lupus anticoagulant was positive. Subsequently, secondary eyeball bleeding, lumbar pain, fever, and other symptoms occurred.

Results: Ultimately, a diagnosis of SLE was made based on his autoantibody results and other tests. Following the administration of methylprednisolone, blood coagulation gradually improved, and no further bleeding was observed.

Discussion: In this report, plasma-mixing studies demonstrated inhibitory and co-factor effects, providing a basis for the disease's subsequent diagnosis and treatment in this patient. This case highlights the laboratory test results analyzed and the reference values for clinical diagnosis and treatment.

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