The TEMPI syndrome is a rare and acquired disorder characterized by five salient features: (1) telangiectasias, (2) elevated erythropoietin and erythrocytosis, (3) monoclonal gammopathy, (4) perinephric fluid collections and (5) intrapulmonary shunting. Complete resolution of symptoms following treatment with plasma cell-directed therapy supports the hypothesis that that monoclonal antibody is causal and pathogenic. Understanding the basis of the TEMPI syndrome will depend on the identification of additional patients and a coordinated international effort.

中文翻译：

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TEMPI 综合征

TEMPI 综合征是一种罕见的获得性疾病，具有五个显着特征：(1) 毛细血管扩张，(2) 红细胞生成素和红细胞增多症，(3) 单克隆丙种球蛋白病，(4) 肾周积液和 (5) 肺内分流。用浆细胞定向疗法治疗后症状完全消退，支持单克隆抗体具有致病性和致病性的假设。了解 TEMPI 综合征的基础将取决于对其他患者的识别和协调的国际努力。