Akioka Y, Hattori M, Konomoto T, Takahashi K, Nagafuchi H, Shiraga H, Kawaguchi H, Ito K
Department of Pediatric Nephrology, Tokyo Women's Medical College, Japan.
Ther Apher. 1998 Aug;2(3):236-9. doi: 10.1111/j.1744-9987.1998.tb00110.x.
We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von Willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.
我们报告一例22岁患有抗磷脂抗体综合征（APS）且合并系统性红斑狼疮的女性患者，其采用冷上清血浆置换有效，改善了腿部难治性静脉血栓形成及复发性血小板减少症。肾活检标本不仅显示有活动性狼疮性肾炎的特征，还显示有肾小动脉血栓形成，后者被认为是血栓性微血管病（TMA）的一种类型。由于在包括血栓性血小板减少性紫癜在内的TMA患者中已报道了超大血管性血友病因子（vWF）多聚体的病理作用，因此使用不含超大vWF多聚体的冷上清进行置换的血浆置换可能是治疗表现为TMA的难治性和慢性复发性APS患者的一种治疗方式选择。
