al-Shahi R, Mason J C, Rao R, Hurd C, Thompson E M, Haskard D O, Davies K A
Br J Rheumatol. 1997 Jul;36(7):794-8. doi: 10.1093/rheumatology/36.7.794.
Thrombocytopenia in patients with acute systemic lupus erythematosus (SLE) frequently presents the clinician with considerable diagnostic and therapeutic difficulties. In this Grand Round, we present a 48-yr-old woman with a 7 yr history of lupus, who presented with acute proliferative glomerulonephritis and nephrotic syndrome, pneumonia, profound hypocomplementaemia and a severe microangiopathic haemolytic anaemia with associated thrombocytopenia. Her thrombocytopenia proved initially refractory to conventional immunosuppressive therapy, and corticosteroids, and resolved only with plasma exchange and repeated fresh frozen plasma infusions. Serological testing revealed high-titre antinuclear antibodies (ANA) and markedly raised antibodies to double-stranded (ds) DNA, but no significant elevation in anticardiolipin antibodies. Platelet-associated IgG and IgM and antibodies to the CD36 glycoprotein antigen, expressed on platelets and endothelium, were detected in the serum. We address some of the difficult diagnostic and management issues raised by this complex patient and the possible immunopathological links between antibodies to CD36, immune-mediated red cell destruction, thrombocytopenia and thrombotic microangiopathic haemolytic anaemia.
急性系统性红斑狼疮（SLE）患者的血小板减少症常常给临床医生带来相当大的诊断和治疗困难。在本次大查房中，我们介绍一位48岁、有7年狼疮病史的女性患者，她表现为急性增殖性肾小球肾炎和肾病综合征、肺炎、严重补体血症以及伴有血小板减少症的严重微血管病性溶血性贫血。她的血小板减少症最初对传统免疫抑制治疗和皮质类固醇治疗无效，仅通过血浆置换和反复输注新鲜冷冻血浆才得以缓解。血清学检测显示高滴度抗核抗体（ANA）以及抗双链（ds）DNA抗体显著升高，但抗心磷脂抗体无明显升高。在血清中检测到血小板相关IgG和IgM以及针对血小板和内皮细胞上表达的CD36糖蛋白抗原的抗体。我们探讨了这位复杂患者所引发的一些诊断和管理难题，以及针对CD36的抗体、免疫介导的红细胞破坏、血小板减少症和血栓性微血管病性溶血性贫血之间可能存在的免疫病理联系。
