儿童低凝血酶原血症-狼疮抗凝物综合征2例报告并文献复习

摘要

To review the clinical features of hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) in children, so as to improve the understanding of the disease. Methods Two cases of HLAS inpatients and 27 cases searched from MEDLINE were retrospectively analyzed, and whose clinical features were summarized. Results The HLAS is more common in female and may be secondary to virus infection (14 cases, 48.2%) or systemic lupus erythematosus (SLE, 14 cases, 48.2%). The most common presenting symptoms were different degrees of bleeding, mainly moderate to severe bleeding (25 cases, 86.2%). The severity of hemorrhage had no correlation with the pathogeny of HLAS or the prothrombin activity. Among 29 patients, 12 patients (41.3%) recovered spontaneously with no special treatment and 15 patients (51.7%) relieved by conservative treatment or immunotherapy, but 2 patients (6.9%) had poor therapeutic effect after treatment. Conclusions HLAS is extremely rare in children and usually associated with virus infection and SLE. The most common presenting symptoms were hemorrhage and hypoprothrombinemia. Most patients have a good prognosis. It is helpful to understand clinical features and diagnostic process of HLAS for the early accurate diagnosis.%目的 对儿童低凝血酶原血症-狼疮抗凝物综合征(hypoprothrombinemia-lupus anticoagulant syndrome,HLAS)的临床特点进行总结,以提高对该病的认识.方法 回顾性分析收治的2例HLAS患儿及医学引文索引(Medline)检索到的27例HLAS患儿的临床资料,总结其临床特点.结果 HLAS女性多发；可继发于病毒感染(14/29,48.2％)或系统性红斑狼疮(14/29,48.2％)；临床主要表现为不同程度的出血,以中重度出血多见(25/29,86.2％),出血严重程度与原发病及凝血酶原活性无明显相关性；29例HLAS患儿中未经特殊治疗缓解者12例(41.4％),经过替代和(或)免疫性治疗缓解者15例(51.7％),病情仍有波动2例(6.9％).结论 HLAS极其罕见,常继发于病毒感染或系统性红斑狼疮,临床表现为出血及低凝血酶原血症,大部分患儿预后良好.掌握其临床特点及诊断流程有助于早期诊断,避免误诊.