TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis
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 rari...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-05-01
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| Series: | Frontiers in Endocrinology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2022.886961/full |
| _version_ | 1818210795911118848 |
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| author | Jian Xu Wenqi Liu Fengjuan Fan Bo Zhang Fei Zhao Yu Hu Chunyan Sun |
| author_facet | Jian Xu Wenqi Liu Fengjuan Fan Bo Zhang Fei Zhao Yu Hu Chunyan Sun |
| author_sort | Jian Xu |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T05:22:17Z |
| format | Article |
| id | doaj.art-2e38d0411fe34bf282dd96ee5be87085 |
| institution | Directory Open Access Journal |
| issn | 1664-2392 |
| language | English |
| last_indexed | 2024-12-12T05:22:17Z |
| publishDate | 2022-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Endocrinology |
| spelling | doaj.art-2e38d0411fe34bf282dd96ee5be870852022-12-22T00:36:34ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922022-05-011310.3389/fendo.2022.886961886961TEMPI Syndrome: Update on Clinical Features, Management, and PathogenesisJian XuWenqi LiuFengjuan FanBo ZhangFei ZhaoYu HuChunyan SunTEMPI (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.https://www.frontiersin.org/articles/10.3389/fendo.2022.886961/fullTEMPI syndromemonoclonal gammopathy of clinical significanceclinical manifestationsplasma cell-directed treatmentpathogenesis |
| spellingShingle | Jian Xu Wenqi Liu Fengjuan Fan Bo Zhang Fei Zhao Yu Hu Chunyan Sun TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis Frontiers in Endocrinology TEMPI syndrome monoclonal gammopathy of clinical significance clinical manifestations plasma cell-directed treatment pathogenesis |
| title | TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis |
| title_full | TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis |
| title_fullStr | TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis |
| title_full_unstemmed | TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis |
| title_short | TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis |
| title_sort | tempi syndrome update on clinical features management and pathogenesis |
| topic | TEMPI syndrome monoclonal gammopathy of clinical significance clinical manifestations plasma cell-directed treatment pathogenesis |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2022.886961/full |
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