红细胞增多、双侧肾周积液伴单克隆免疫球蛋白血症

A case of erythrocytosis,perinephric fluid collections and monoclonal gammopathy

老年男性,病程3年余,体检发现双侧胸腔积液,伴前胸部皮肤毛细血管扩张,1年后发现双侧胸腔、腹腔和盆腔积液,红细胞增多,血红细胞生成素(EPO)水平升高,血免疫固定电泳阳性,骨髓细胞学未见明显异常,外院诊断“真性红细胞增多症”,给予羟基脲治疗,血红蛋白控制在正常范围。1月前发现双侧肾周大量积液,腹壁皮肤脂肪活检示刚果红染色阳性,考虑为系统性淀粉样变性。至国家肾脏疾病临床医学研究中心重复腹壁皮肤脂肪活检示刚果红染色阴性,免疫固定电泳图谱见κ型IgG单克隆免疫球蛋白条带,骨髓流式示0.3%异常克隆浆细胞,右心声学造影提示小肺动静脉分流,明确诊断为TEMPI综合征。接受8个疗程硼替佐米+地塞米松化疗后,血清免疫固定电泳转阴,EPO降至正常范围,皮肤毛细血管扩张基本消失,肾周积液明显减少。

A 71-year-old man presented with bilateral pleural effusion and telangiectasia for more than 3 years.One year later,bilateral pleural,abdominal and pelvic effusion were showed on PET-CT.Erythrocytosis with high erythropoietin(EPO),and monoclonal gammopathy were found,while bone marrow cytology showed no obvious abnormity.He was diagnosed with polycythemia vera and received long-term treatment with hydroxyurea.Bilateral perirenal effusion was found 3 years after the onset of the disease.The subcutaneous fat biopsy showed Congo red staining positive,and systemic amyloidosis was considered at another hospital.After admission to our hospital,serum immunofixation showed IgGκmonoclonal protein,bone marrow flow cytometry showed 0.3%abnormal clonal plasma cells,and right heart contrast echocardiography suggested arteriovenous shunting.The diagnosis of TEMPI syndrome was confirmed,and he subsequently received a total of eight cycles of bortezomib and dexamethasone,after which his serum immunofixation electrophoresis turned negative,EPO level decreased to normal,skin telangiectasis and perirenal fluid improved significantly.