儿童抗磷脂抗体阳性13例报告

目的 探讨儿科抗磷脂抗体(antiphospholipid antibodies,APL)阳性病例的特点,以提高临床诊治水平。方法 2000～2002于我科就诊的13例APL阳性的病例,6～13岁,男7例,女6例,对其临床资料进行总结分析。结果 (1)13例中有8例原发病为系统性红斑狼疮(systemic lupus erythematosus,SLE),2例原发病为急性链球菌感染,3例未找到原发病因,考虑为原发APL。(2)本组原发病为SLE的8例患儿抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic autoantibodies,ANCA)均为阴性。(3)对8例APL阳性伴血小板减少,皮肤黏膜瘀点、瘀斑的病例,给予大剂量丙种球蛋白(WIG)静脉滴注,进行免疫调节治疗;对5例APL阳性伴血管血栓、血栓血管炎症状者,则积极进行抗凝、抗栓治疗,多取得较好的临床疗效。结论 儿科APL阳性病例以继发性多见,SLE是最多见的原发疾病。APL阳性可以多种疾病形式出现。对APL阳性的患儿应区别对待,根据不同病因、病情分别进行以抗凝、抗栓为主或以免疫调节为主的治疗,可以尽快的改善临床症状,改善预后。

Clinical significance of antiphospholipid antibody in pediatric patients and review of literature

OBJECTIVE: Antiphospholipid antibody (APL) is a particularly important laboratory diagnostic criterion for antiphospholipid syndrome (APS). The significances of positive APL in childhood are seldom reported nor fully understood. The purpose of this study was to analyze 13 cases with positive APL seen in our hospital and to study the relationship between the positive rates of APL and various clinical diseases especially systemic lupus erythematosus (SLE) in order to improve the clinical diagnoses and treatment level of APS in children. METHODS: The clinical data collected from 2000 to 2002 of 13 hospitalized children with positive APL were retrospectively evaluated. Enzyme linked immunosorbent assay (ELISA) and indirect immunofluorescence technique were used respectively to detect APL and antineutrophil cytoplasmic autoantibodies (ANCA) of sera from those children. Other various indexes were also detected according to different characteristics of different diseases. RESULTS: Eight cases had SLE; 2 had acute post-streptococcal infections. The other 3 cases did not show any evidences of primary diseases; they probably had primary APS. SLE was the most common primary diseases to cause development of APL and the cases with SLE showed more severe cutaneous vasculitis than SLE patients who were negative for APL. There was no significant relationship between the positive rates of APL and that of ANCA. Eight APL positive cases complicated with thrombocytopenia and bleeding were treated with high dosage of immunoglobulin [400 mg/(kg.d), for 3 - 5 d] intravenously; the clinical conditions of these cases were ameliorated soon. While the 5 cases who had thrombotic vasculitis and thromboembolism were treated with anticoagulant and antithrombotic therapy with low molecular weight heparin [50 - 100 U/(kg.d)], which led to good clinical effects. CONCLUSIONS: The clinical manifestations of children positive for APL were somehow different from those of adults. Positive APL itself may be nonspecific, it can occur from different causes of diseases. APL detection may be useful to suggest anticoagulant and/or antithrombosis therapy. Treatments for APS should be variable according to different causes and severity of diseases, in the cases of thrombocytopenia and bleeding, high dose intravenous immunoglobulin should be given as soon as possible, while in the cases of thrombotic vasculitis and thromboembolism, anticoagulant and antithrombotic therapy should be given soon.