儿科原发性抗磷脂综合征：超越血栓。32 例报告及证据回顾。
Primary antiphospholipid syndrome in pediatrics: beyond thrombosis. Report of 32 cases and review of the evidence.
机构信息
Department of Pediatric Rheumatology, National Medical Center La Raza, IMSS, Vallejo y Jacarandas, colonia La Raza, Azcapotzalco, D.F. México CP, 02990, México City, México.
Department of Pediatric Hematology, National Medical Center La Raza, IMSS, Mexico City, México.
出版信息
Pediatr Rheumatol Online J. 2022 Feb 14;20(1):13. doi: 10.1186/s12969-022-00673-y.
OBJECTIVE
Describe the frequency of thrombotic and non-thrombotic clinical manifestations, laboratory, treatment and prognosis in patients with pediatric primary antiphospholipid syndrome.
MATERIAL AND METHODS
A retrospective study was carried out in patients with a diagnosis of primary antiphospholipid antibody syndrome, under 16 years of age, under follow-up by the pediatric rheumatology service of the General Hospital, National Medical Center, La Raza, from January 2013 to December 2020. The antiphospholipid syndrome was defined when it met the laboratory criteria of the Sidney criteria and the presence of thrombosis or non-criteria manifestations of the disease (hematological, neurological, cutaneous, renal, cardiac or pulmonary). Demographic, clinical, laboratory, treatment, and prognosis data were collected.
RESULTS
We report 32 patients, 21 female (65%) and 11 male (35%), mean age 11.75 years, evolution time 16 weeks. Thrombosis 9 patients (28%), 1 arterial and 8 venous. Non-thrombotic manifestations; Hematologic: thrombocytopenia 22 patients (69%), autoimmune hemolytic anemia 13 (40%), Fisher-Evans syndrome 6 (19%), lupus anticoagulant with hypoprothrombinemia syndrome 2 (6%). Dermatological: livedo reticularis 20 (62%), skin ulcers 2 (6%), Raynaud's phenomenon 8 (25%). Neurological: epilepsy 1 (3%), migraine 3 (9%), chorea 1 (3%) and cognitive impairment 3 (9%). Renal in 4 (13%). Laboratory: prolonged aPTT 30 (93%), lupus anticoagulant 32 (100%), positive IgG anticardiolipin 20 (62%), positive IgM anticardiolipin 19 (60%). AntiB2GPI was performed in only 3 patients, being positive in all.
TREATMENT
anticoagulation in patients with thrombosis, antiplatelet in 23 (72%), steroid 30 (94%), immunosuppressant 30 (94%) and rituximab 4 (12.5%). No deaths were reported.
CONCLUSIONS
The clinical characteristics of patients with pediatric primary antiphospholipid syndrome differ from those presented in adults, since non-thrombotic manifestations are more frequent in children, for which classification criteria that include these manifestations are necessary for a better characterization of the disease in pediatric population.
目的
描述儿科原发性抗磷脂综合征患者血栓形成和非血栓形成的临床表现、实验室检查、治疗和预后的频率。
材料和方法
对 2013 年 1 月至 2020 年 12 月期间在国家医疗中心拉扎（General Hospital, National Medical Center, La Raza）儿科风湿病服务处接受随访的年龄在 16 岁以下的原发性抗磷脂抗体综合征患者进行回顾性研究。当符合 Sidney 标准的实验室标准和血栓形成或疾病的非标准表现（血液、神经、皮肤、肾脏、心脏或肺部）时，诊断为抗磷脂综合征。收集人口统计学、临床、实验室、治疗和预后数据。
结果
我们报告了 32 名患者，21 名女性（65%）和 11 名男性（35%），平均年龄 11.75 岁，病程 16 周。9 名患者（28%）发生血栓形成，1 例动脉血栓形成，8 例静脉血栓形成。非血栓形成表现：血液学：血小板减少 22 例（69%），自身免疫性溶血性贫血 13 例（40%），Fisher-Evans 综合征 6 例（19%），低蛋白血症伴狼疮抗凝物综合征 2 例（6%）。皮肤：网状青斑 20 例（62%），皮肤溃疡 2 例（6%），雷诺现象 8 例（25%）。神经：癫痫 1 例（3%），偏头痛 3 例（9%），舞蹈病 1 例（3%），认知障碍 3 例（9%）。肾脏受累 4 例（13%）。实验室：延长 aPTT30 例（93%），狼疮抗凝物 32 例（100%），IgG 型抗心磷脂抗体阳性 20 例（62%），IgM 型抗心磷脂抗体阳性 19 例（60%）。仅对 3 例患者进行了抗 B2GPI 检测，均为阳性。
治疗
血栓形成患者抗凝，23 例（72%）抗血小板，30 例（94%）类固醇，30 例（94%）免疫抑制剂，4 例（12.5%）利妥昔单抗。未报告死亡。
结论
儿科原发性抗磷脂综合征患者的临床特征与成人不同，因为儿童中更常见非血栓形成表现，因此需要包含这些表现的分类标准，以便更好地描述儿科人群中的疾病特征。
Zotero 插件