儿童低凝血酶原血症–狼疮抗凝物综合征：临床特征的分析及探讨

Analysis and Exploration of Clinical Features of Children with Low Prothrombinemia Lupus Anticoagulant Syndrome

摘要: 目的：研究低凝血酶原血症–狼疮抗凝物综合征(HLAS)患儿的临床特点，以提高儿科医师对此病的认识，在面对危急出血倾向或症状时尽早认识并及时诊断，启动适当的管理和干预，避免误诊，以减少发生危及生命的并发症的危险。方法：回顾性分析我院诊治的3例HLAS患儿，并结合文献报道加以归纳总结。结果：报道的3例HLAS患儿，其中2例合并活动性出血症状，当中1例患儿多次出现凝血指标的危急值，并且合并了出血及大剂量甲泼尼龙冲击治疗不耐受的危急症状。结论：HLAS是SLE患者出血的罕见原因，在评估SLE儿童时需警惕。当SLE患儿存在PT和aAPTT的延长，没有任何脓毒症、DIC或巨噬细胞活化综合征的证据时则提示该病。因此，对于患有SLE的患儿且合并了LA抗体阳性，尤其存在出血倾向或症状，需监测患儿的凝血功能及凝血因子活性，早期识别HLAS，尽早使用免疫抑制治疗，为大多数患儿提供了良好的预后，避免出现危及生命的出血症状及脏器损伤。而不合并SLE的HLAS，无活动性出血表现，需定期随访，警惕后续合并SLE。

Abstract: Objective: To study the clinical characteristics of children with low prothrombin associated lupus anticoagulant syndrome (HLAS), in order to improve pediatricians’ understanding of this disease, recognize and diagnose critical bleeding tendencies or symptoms as soon as possible, initiate ap-propriate management and intervention, avoid misdiagnosis, and reduce the risk of life-threatening complications. Method: A retrospective analysis was conducted on 3 cases of HLAS diagnosed and treated in our hospital, and literature reports were summarized and summarized. Results: Among the 3 reported cases of HLAS, 2 cases had symptoms of active bleeding, and 1 case had multiple critical values of coagulation indicators, as well as severe symptoms of bleeding and intolerance to high-dose methylprednisolone pulse therapy. Conclusion: HLAS is a rare cause of bleeding in SLE patients, and caution should be exercised when evaluating children with SLE. When SLE patients have prolonged PT and aAPTT without any evidence of sepsis, DIC, or macrophage activation syn-drome, it indicates the disease. Therefore, for children with SLE who have a positive LA antibody, especially those with bleeding tendencies or symptoms, it is necessary to monitor their coagulation function and coagulation factor activity, identify HLAS early, and use immunosuppressive therapy as early as possible, providing a good prognosis for most children and avoiding life-threatening bleeding symptoms and organ damage. HLAS without SLE has no active bleeding manifestations and requires regular follow-up to be vigilant for subsequent SLE complications.