关键词: TEMPI综合征,红细胞增多,单克隆免疫球蛋白血症,毛细血管扩张,肾周积液

Abstract: 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 IgG κ monoclonal 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.

Key words: TEMPI syndrome, erythrocytosis, monoclonal gammopathy, telangiectasiaperinephric fluid