Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Endocrinol (Lausanne). 2022 May 19;13:886961. doi: 10.3389/fendo.2022.886961. eCollection 2022.
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.
TEMPI（毛细血管扩张、红细胞生成素水平升高和红细胞增多症、单克隆丙种球蛋白病、肾周液体积聚和肺内分流）综合征是一种罕见的新定义的多系统疾病，属于“具有临床意义的单克隆丙种球蛋白病”。由于其罕见性，该疾病的病因、发病机制和临床特征在很大程度上尚不清楚。由于其隐匿和多样的临床表现，漏诊和误诊很常见。近年来，随着越来越多的患者（包括三例死亡病例）被确诊，除了 TEMPI 综合征的典型五联征外，还报道了一些其他特殊的临床表现。同时，也进行了一些试图确定 TEMPI 综合征发病机制的研究。在这篇综述中，我们总结了报告的 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选择，包括复发/难治性疾病患者。此外，我们还概述了 TEMPI 综合征病理生理学的现有知识。
