Department of Hematology, Erasmus MC University Medical Center, Rotterdam, the Netherlands.
Department of Hematology, Erasmus MC University Medical Center, Rotterdam, the Netherlands.
Transfus Apher Sci. 2021 Oct;60(5):103192. doi: 10.1016/j.transci.2021.103192. Epub 2021 Jun 15.
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by clinical findings including thrombosis and/or obstetric complication and laboratory findings, e.g. ≥1 positive antiphospholipid antibodies (aPL) (lupus anticoagulant, anticardiolipin IgG/IgM and/or anti-β2-glycoprotein IgG/IgM). A rare APS clinical entity is severe necrosis which is difficult to treat and often does not respond to anticoagulant therapy. Three consecutive patients with primary or secondary APS who presented with necrotic skin lesions secondary to APS were treated with therapeutic plasma exchange (TPE), glucocorticoids and low-molecular-weight heparin. All patients had a rapid-onset, either full or significant recovery of their APS-related necrotic lesions. Upon treatment, one patients showed resolution of lupus anticoagulant. Two patients had a decrease of at least 88 % in aPL titers after the initial treatment, and were kept on TPE maintenance every 5-6 weeks. None of the patients experienced significant side effects of the TPE. This is the first case series showing the clinical benefits of TPE in patients with ischemic and necrotic skin lesions due to severe anticoagulant-refractory vascular APS.
抗磷脂综合征（APS）是一种系统性自身免疫性疾病，其临床特征包括血栓形成和/或产科并发症以及实验室检查结果，例如≥1 种抗磷脂抗体（aPL）阳性（狼疮抗凝物、抗心磷脂 IgG/IgM 和/或抗β2-糖蛋白 IgG/IgM）。APS 的一种罕见临床实体是严重坏死，这种疾病难以治疗，且通常对抗凝治疗无反应。连续 3 例原发性或继发性 APS 患者因 APS 出现皮肤坏死性病变，接受了治疗性血浆置换（TPE）、糖皮质激素和低分子肝素治疗。所有患者的 APS 相关坏死性病变均迅速出现，要么完全恢复，要么显著恢复。在治疗过程中，1 例患者狼疮抗凝物得到了缓解。2 例患者在初始治疗后 aPL 滴度至少下降了 88%，并每 5-6 周接受一次 TPE 维持治疗。所有患者均未出现 TPE 的明显副作用。这是首个病例系列报告，表明 TPE 对严重抗凝难治性血管 APS 引起的缺血性和坏死性皮肤病变患者具有临床益处。
