Department of Anesthesiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdansantiao Street, Dongcheng District, Beijing, 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdansantiao Street, Dongcheng District, Beijing, 100730, China.
Clin Rheumatol. 2018 May;37(5):1421-1426. doi: 10.1007/s10067-017-3912-2. Epub 2017 Nov 13.
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers. TTP was thus diagnosed. The patient also had positive anti-nuclear antibody, anti-SSA, and anti-SSB; however, anti-double stranded DNA (dsDNA) was negative. These drove us to perform ocular and dental sicca evaluation and the finial diagnosis was TTP secondary to SS. Plasma exchange and corticosteroid therapy were effective to control TTP. Cyclophosphamide was subsequently added when the platelet count was stable. The total duration of corticosteroid and cyclophosphamide was 8 and 6 months, respectively. The patient recovered without relapse at 1-year follow-up. To our knowledge, this was the first case of SS initially presented as TTP in a male patient. The case also elucidated the importance of autoantibody screen in the workup of TTP and the benefits of adjunctive immunosuppressive therapy in relapse prevention.
血栓性血小板减少性紫癜（TTP）是一种潜在致命的多系统疾病，可能由自身免疫性疾病引起。然而，TTP 与干燥综合征（SS）同时发生的情况极为罕见，尤其是在男性患者中。一位 56 岁的中国男性因弥漫性瘀斑就诊。随后他逐渐出现一过性言语不清、进行性意识混乱、激动、四肢无力和发热。实验室检查提示贫血、血小板减少、乳酸脱氢酶显著升高、外周血涂片出现裂片红细胞、以及去整合素样金属蛋白酶与血小板反应蛋白 13 型（ADAMTS13）活性缺乏伴高抑制剂滴度。因此诊断为 TTP。患者还存在抗核抗体、抗 SSA 和抗 SSB 阳性，但抗双链 DNA（dsDNA）阴性。这些促使我们进行眼和口腔干燥评估，最终诊断为继发于 SS 的 TTP。血浆置换和皮质类固醇治疗有效控制了 TTP。当血小板计数稳定后，随后加用环磷酰胺。皮质类固醇和环磷酰胺的总疗程分别为 8 个月和 6 个月。患者在 1 年随访时无复发而痊愈。据我们所知，这是首例男性患者以 TTP 首发的 SS 病例。该病例还阐明了在 TTP 诊断中进行自身抗体筛查的重要性，以及辅助免疫抑制治疗在预防复发中的益处。
