The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-0855, Japan.
Mod Rheumatol. 2012 Sep;22(5):769-73. doi: 10.1007/s10165-011-0563-z. Epub 2011 Nov 29.
A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β(2)-glycoprotein I (β(2)GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β(2)GPI might be associated with the radical manifestation of CAPS.
一名 16 岁男性患有严重血小板减少症和进行性多器官梗死，被诊断为伴有系统性红斑狼疮的灾难性抗磷脂综合征（CAPS），并通过抗凝剂、皮质类固醇、血浆置换和静脉注射环磷酰胺联合成功治疗。同时检测到抗磷脂酰丝氨酸/凝血酶原（PS/PT）复合物和心磷脂（CL）/β(2)-糖蛋白 I（β(2)GPI）抗体，表明 PS/PT 和 CL/β(2)GPI 的不同途径可能与 CAPS 的激进表现有关。
