Department of Medicine, University of Toronto, Toronto, Ontario, Canada
Department of Medicine, Division of Nephrology, University of Toronto, Toronto, Ontario, Canada.
BMJ Case Rep. 2020 Nov 23;13(11):e236654. doi: 10.1136/bcr-2020-236654.
A previously healthy 29-year-old man initially presented to the hospital with pleuritic chest pain and shortness of breath. Over the next 2 months he developed ongoing fevers and night sweats with recurrent exudative pleural effusions and ascites. He had an extensive infectious and autoimmune workup that was unremarkable. He had an initial lymph node biopsy that showed reactive changes only. He had an acute kidney injury and his renal biopsy revealed thrombotic microangiopathy. His liver biopsy showed non-specific inflammatory changes. His bone marrow biopsy showed megakaryocyte hyperplasia and fibrosis, which raised suspicion for the thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction and organomegaly syndrome subtype of multicentric Castleman disease. This prompted a repeat lymph node biopsy, showing changes consistent with mixed type Castleman disease that fit with his clinical picture. He was initiated on steroids and siltuximab with significant clinical improvement.
一位既往健康的 29 岁男性最初因胸痛和呼吸急促到医院就诊。在接下来的 2 个月中,他持续发热和盗汗,反复出现渗出性胸腔积液和腹水。他进行了广泛的感染和自身免疫检查,但均未见异常。最初的淋巴结活检仅显示反应性改变。他出现急性肾损伤,肾脏活检显示血栓性微血管病。肝脏活检显示非特异性炎症改变。骨髓活检显示巨核细胞增生和纤维化,这提示他患有特发性血小板减少性紫癜、腹水、网状纤维纤维化、肾功能不全和器官肿大综合征型多发性骨髓瘤。这促使他再次进行淋巴结活检,结果显示符合他临床表现的混合型 Castleman 病改变。他开始接受类固醇和西妥昔单抗治疗,临床症状显著改善。