PDF(Pubmed)
Abstract：
TEMPI (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) syndrome is a rare and newly defined multisystemic disease, which belongs to \"monoclonal gammopathy of clinical significances\". Due to its rarity, the etiology, pathogenesis, and clinical features of this disease remain largely unknown. Owing to its hidden and diverse clinical manifestations, missed diagnosis and misdiagnosis are common. In recent years, as more patients (including three fatal cases) were identified, some special clinical manifestations other than the typical pentad of TEMPI syndrome have been reported. Meanwhile, several studies attempting to identify the pathogenesis of TEMPI syndrome were conducted. In this review, we summarize the reported clinical characteristics of TEMPI syndrome and discuss the current and potential treatment options for patients with TEMPI syndrome, including those with relapsed/refractory disease. Furthermore, we provide an overview of current knowledge on the pathophysiology of TEMPI syndrome.
摘要：
TEMPI(毛细血管扩张，促红细胞生成素水平升高和红细胞增多，单克隆丙种球蛋白病，肾周积液集合,肺内分流）综合征是一种罕见且新定义的多系统疾病，属于“临床意义的单克隆丙种球蛋白病”。由于它的稀有性,病因，发病机制，这种疾病的临床特征仍然未知。由于其隐匿多样的临床表现,漏诊和误诊比较常见。近年来,随着更多的患者（包括3例致命病例）被确认，除典型的TEMPI综合征外，一些特殊的临床表现已有报道。同时,进行了几项试图确定TEMPI综合征发病机制的研究.在这次审查中,我们总结了已报道的TEMPI综合征的临床特征，并讨论了TEMPI综合征患者的当前和潜在治疗方案，包括复发/难治性疾病。此外，我们概述了TEMPI综合征病理生理学的现有知识.
