Asano Takeshi, Narazaki Hidehiko, Kaizu Kiyohiko, Kuwabara Kentaroh, Fujino Osamu, Itoh Yasuhiko
Department of Pediatrics, Nippon Medical School Chiba Hokusoh Hospital.
J Nippon Med Sch. 2015;82(2):117-20. doi: 10.1272/jnms.82.117.
Antiphospholipid syndrome (APS) is an autoimmune disease caused by antiphospholipid antibodies. At our institution, APS is diagnosed on the basis of the Sapporo criteria, which consist of thrombosis and recurrent pregnancy-related complications and the following laboratory findings: the presence of lupus anticoagulant, anticardiolipin antibody, or anti-β2 glycoprotein 1 antibody. However, we sometimes treat patients we strongly suspect of having APS but who do not satisfy the laboratory criteria. To accommodate such suspected cases, a subtype of APS termed seronegative APS has been proposed. Here, we report on a man with chronic thromobocytopenic purpura since the age of 3 years and multiple cerebral infarctions since the age of 14 years who finally received a diagnosis of seronegative APS with positive antiphosphatidylethanolamine antibodies.
抗磷脂综合征(APS)是一种由抗磷脂抗体引起的自身免疫性疾病。在我们机构,APS是根据札幌标准进行诊断的,该标准包括血栓形成、与复发性妊娠相关的并发症以及以下实验室检查结果:狼疮抗凝物、抗心磷脂抗体或抗β2糖蛋白1抗体的存在。然而,我们有时会治疗那些我们强烈怀疑患有APS但不符合实验室标准的患者。为了适应此类疑似病例,已提出一种称为血清阴性APS的APS亚型。在此,我们报告一名自3岁起患有慢性血小板减少性紫癜、自14岁起发生多次脑梗死的男性,最终被诊断为抗磷脂酰乙醇胺抗体阳性的血清阴性APS。