Antiphospholipid syndrome, monoclonal gammopathy, and cryoglobulinemia overlap leading to recurrent cutaneous microvascular thrombosis: A case report and retrospective cohort study

EJHaem Pub Date : 2024-07-28 DOI:10.1002/jha2.987
Alexandra Bohm, Bo Angela Wan, Amir Karin, Lauren J. Lee, Agnes Y. Y. Lee, Edward M. Conway, Chieh Min Benjamin Lai
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Abstract

Antiphospholipid syndrome (APS), cryoglobulinemia, and monoclonal gammopathies are variably accompanied by thrombotic complications. We describe a patient with recurrent skin microvascular thrombosis, APS, cryoglobulinemia, marginal zone lymphoma, and IgMκ monoclonal gammopathy, responsive to chemoimmunotherapy. The cryoglobulin fraction contained the IgMκ paraprotein, while antiphospholipid antibodies (aPL) were predominantly in the cryosupernatant. A retrospective analysis of aPL-positive patients in our institution showed that 8.1% co-expressed monoclonal gammopathy. These overlap patients had thrombotic complications and most had recurrences. Patients with multiple gammopathies of thrombotic significance may have several autoantibodies and constitute a high-risk group.

Abstract Image

抗磷脂综合征、单克隆丙种球蛋白病和冷球蛋白血症重叠导致复发性皮肤微血管血栓形成:病例报告和回顾性队列研究
抗磷脂综合征(APS)、低温球蛋白血症和单克隆丙种球蛋白病会不同程度地伴有血栓形成并发症。我们描述了一名患有复发性皮肤微血管血栓、APS、低温球蛋白血症、边缘区淋巴瘤和 IgMκ 单克隆抗体病的患者,该患者对化学免疫疗法有反应。低温球蛋白部分含有 IgMκ 副蛋白,而抗磷脂抗体(aPL)则主要存在于低温上清液中。我院对 aPL 阳性患者的回顾性分析显示,8.1% 的患者同时表达单克隆抗体。这些重叠的患者都有血栓并发症,而且大多数都复发了。具有血栓形成意义的多种丙种球蛋白病患者可能同时存在多种自身抗体,是一个高危群体。
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