Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation
Abstract The TEMPI syndrome is a very rare paraneoplastic syndrome associated with plasma cell dyscrasia and monoclonal gammopathy. First described in 2011, the pathophysiology of TEMPI syndrome is still unknown. Essential for diagnosis is to recognize the five clinical findings: telangiectasias, er...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-11-01
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| Series: | eJHaem |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jha2.561 |
| _version_ | 1827800279676878848 |
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| author | Davide Nappi Martina Tauber David Sykes |
| author_facet | Davide Nappi Martina Tauber David Sykes |
| author_sort | Davide Nappi |
| collection | DOAJ |
| description | Abstract The TEMPI syndrome is a very rare paraneoplastic syndrome associated with plasma cell dyscrasia and monoclonal gammopathy. First described in 2011, the pathophysiology of TEMPI syndrome is still unknown. Essential for diagnosis is to recognize the five clinical findings: telangiectasias, erythrocytosis and elevated serum erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting. Here we report a case of a woman with the coexistence of TEMPI and leukocytoclastic vasculitis, shedding light on a possible common inflammatory pathway involved in the pathogenesis of the syndrome. |
| first_indexed | 2024-03-11T20:06:06Z |
| format | Article |
| id | doaj.art-070fea2566cb4c95a3a027b750eae4c4 |
| institution | Directory Open Access Journal |
| issn | 2688-6146 |
| language | English |
| last_indexed | 2024-03-11T20:06:06Z |
| publishDate | 2022-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | eJHaem |
| spelling | doaj.art-070fea2566cb4c95a3a027b750eae4c42023-10-03T16:50:47ZengWileyeJHaem2688-61462022-11-01341385138710.1002/jha2.561Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantationDavide Nappi0Martina Tauber1David Sykes2Hematology and Bone Marrow Transplantation Bolzano Hospital Bolzano ItalyDepartment of Pathology Bolzano Hospital Bolzano ItalyMassachusetts General Hospital Center for Regenerative Medicine Boston Massachusetts USAAbstract The TEMPI syndrome is a very rare paraneoplastic syndrome associated with plasma cell dyscrasia and monoclonal gammopathy. First described in 2011, the pathophysiology of TEMPI syndrome is still unknown. Essential for diagnosis is to recognize the five clinical findings: telangiectasias, erythrocytosis and elevated serum erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting. Here we report a case of a woman with the coexistence of TEMPI and leukocytoclastic vasculitis, shedding light on a possible common inflammatory pathway involved in the pathogenesis of the syndrome.https://doi.org/10.1002/jha2.561angiogenesiserythrocytosisinflammationMGUSmultiple myeloma |
| spellingShingle | Davide Nappi Martina Tauber David Sykes Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation eJHaem angiogenesis erythrocytosis inflammation MGUS multiple myeloma |
| title | Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| title_full | Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| title_fullStr | Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| title_full_unstemmed | Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| title_short | Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| title_sort | coexistence of tempi syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation |
| topic | angiogenesis erythrocytosis inflammation MGUS multiple myeloma |
| url | https://doi.org/10.1002/jha2.561 |
| work_keys_str_mv | AT davidenappi coexistenceoftempisyndromeandleukocytoclasticvasculitissuccessfullytreatedwithautologousstemcelltransplantation AT martinatauber coexistenceoftempisyndromeandleukocytoclasticvasculitissuccessfullytreatedwithautologousstemcelltransplantation AT davidsykes coexistenceoftempisyndromeandleukocytoclasticvasculitissuccessfullytreatedwithautologousstemcelltransplantation |