Matsumura Gai, Ohno Sayaka, Hasegawa Shun, Shimizu Ryo
Department of Hematology, Asahi General Hospital, Chiba, JPN.
Department of Clinical Pathology, Asahi General Hospital, Chiba, JPN.
Cureus. 2025 Aug 28;17(8):e91158. doi: 10.7759/cureus.91158. eCollection 2025 Aug.
Nodal marginal zone lymphoma (NMZL) is an uncommon, mature B-cell lymphoma, rarely associated with acquired von Willebrand syndrome (AVWS), a bleeding disorder caused by von Willebrand factor (VWF) defects secondary to lymphoproliferative disorders (LPDs). We report a 68-year-old woman with longstanding Sjögren's syndrome, presenting with persistent oral bleeding, anemia, prolonged activated partial thromboplastin time (APTT, 50.5 seconds), and IgA-λ monoclonal gammopathy. Further investigation revealed severely decreased VWF ristocetin cofactor activity (VWF:RCo, 16%). Imaging revealed systemic lymphadenopathy, and a lymph node biopsy confirmed NMZL. The patient was treated with four weekly doses of rituximab, resulting in cessation of bleeding, improvement of coagulation parameters, resolution of anemia, and significant lymphadenopathy regression - consistent with complete response by computed tomography (CT) per Lugano 2014. This case highlights the diagnostic challenges of AVWS associated with NMZL and demonstrates rituximab's efficacy in treating both lymphoma and AVWS.
结外边缘区淋巴瘤（NMZL）是一种罕见的成熟B细胞淋巴瘤，很少与获得性血管性血友病综合征（AVWS）相关，AVWS是一种由淋巴增殖性疾病（LPDs）继发的血管性血友病因子（VWF）缺陷引起的出血性疾病。我们报告一名68岁患有长期干燥综合征的女性，出现持续性口腔出血、贫血、活化部分凝血活酶时间延长（APTT，50.5秒）和IgA-λ单克隆丙种球蛋白病。进一步检查发现血管性血友病因子瑞斯托霉素辅因子活性严重降低（VWF:RCo，16%）。影像学检查显示全身淋巴结病，淋巴结活检确诊为NMZL。该患者接受了四周的利妥昔单抗治疗，出血停止，凝血参数改善，贫血得到缓解，淋巴结病明显消退——根据2014年卢加诺标准，计算机断层扫描（CT）显示完全缓解。该病例突出了与NMZL相关的AVWS的诊断挑战，并证明了利妥昔单抗在治疗淋巴瘤和AVWS方面的疗效。
