The patients were all diagnosed at middle age. A monoclonal gammopathy was implicated in all patients tested.[1]
Causeedit
The cause of the syndrome is unknown. The abnormal plasma-cell clone and/or the monoclonal gammopathy are suggested to be triggers of the disease.[2]
Diagnosisedit
The diagnosis is based on the five characteristics described above.[2]
Treatmentedit
Complete and partial disappearance of the symptoms of the TEMPI syndrome was reported with the drugs bortezomib,[3]daratumumab[4] and autologous stem cell transplantation.[5]
Historyedit
In 2010, the case of a man with unexplained erythrocytosis and perinephric fluid collection as main features was described in the Case Records of the Massachusetts General Hospital.[6]
Referencesedit
^Sykes, David B.; Schroyens, Wilfried; O'Connell, Casey (2011). "TEMPI Syndrome – A Novel Multisystem Disease". N Engl J Med. 365 (5): 475–477. doi:10.1056/NEJMc1106670. PMID 21812700. S2CID 35990145.
^ abSykes, David B.; O'Connell, Casey; Schroyens, Wilfried (2020-04-09). "The TEMPI syndrome". Blood. 135 (15): 1199–1203. doi:10.1182/blood.2019004216. ISSN 1528-0020. PMID 32108223.
^Schroyens, Wilfried; O'Connell, Casey; Sykes, David B. (2012). "Complete and Partial Responses of the TEMPI Syndrome to Bortezomib" (PDF). N Engl J Med. 367 (8): 778–780. doi:10.1056/NEJMc1205806. PMID 22913703.
^Sykes, David B.; Schroyens, W. (2018). "Complete Responses in the TEMPI Syndrome after Treatment with Daratumumab". N Engl J Med. 378 (23): 2240–2242. doi:10.1056/NEJMc1804415. PMID 29874534. S2CID 205064988.
^Kenderian, S.S..; Rosado, F.G; Sykes, D.B.; Hoyer, J.D.; Lacy, M.Q. (2015). "Long-term complete clinical and hematological responses of the TEMPI syndrome after autologous stem cell transplantation". Leukemia. 29 (12): 2414–2416. doi:10.1038/leu.2015.298. PMID 26500143.
^Bazari, Hasan; Attar, Eyal C.; Dahl, Douglas M.; Uppot, Raul N.; Colvin, Robert B. (2010). "Case Records of the Massachusetts General Hospital. Case 23-2010: A 49-Year-Old Man with Erythrocytosis, Perinephric Fluid Collections, and Renal Failure". N Engl J Med. 363 (5): 463–475. doi:10.1056/NEJMcpc1004086. PMID 20818867.
External linksedit
Office of Rare Diseases Research (US)
Orpha.net (EU)
Hematopia TEMPI Page
April 12, 2024
tempi, syndrome, orphan, disease, where, patients, share, five, characteristics, from, which, acronym, derived, telangiectasias, elevated, erythropoietin, erythrocytosis, monoclonal, gammopathy, perinephric, fluid, collection, intrapulmonary, shunting, other, . TEMPI syndrome is an orphan disease where the patients share five characteristics from which the acronym is derived telangiectasias elevated erythropoietin and erythrocytosis monoclonal gammopathy perinephric fluid collection and intrapulmonary shunting TEMPI syndromeOther namesTelangiectasia erythrocytosis monoclonal gammopathy perinephric fluid collections intrapulmonary shunting syndrome Contents 1 Signs and symptoms 2 Cause 3 Diagnosis 4 Treatment 5 History 6 References 7 External linksSigns and symptoms editTEMPI SymptomT TelangiectasiasE Elevated Erythropoietin and ErythrocytosisM Monoclonal gammopathyP Perinephric fluid collectionsI Intrapulmonary shuntingThe patients were all diagnosed at middle age A monoclonal gammopathy was implicated in all patients tested 1 Cause editThe cause of the syndrome is unknown The abnormal plasma cell clone and or the monoclonal gammopathy are suggested to be triggers of the disease 2 Diagnosis editThe diagnosis is based on the five characteristics described above 2 Treatment editComplete and partial disappearance of the symptoms of the TEMPI syndrome was reported with the drugs bortezomib 3 daratumumab 4 and autologous stem cell transplantation 5 History editIn 2010 the case of a man with unexplained erythrocytosis and perinephric fluid collection as main features was described in the Case Records of the Massachusetts General Hospital 6 References edit Sykes David B Schroyens Wilfried O Connell Casey 2011 TEMPI Syndrome A Novel Multisystem Disease N Engl J Med 365 5 475 477 doi 10 1056 NEJMc1106670 PMID 21812700 S2CID 35990145 a b Sykes David B O Connell Casey Schroyens Wilfried 2020 04 09 The TEMPI syndrome Blood 135 15 1199 1203 doi 10 1182 blood 2019004216 ISSN 1528 0020 PMID 32108223 Schroyens Wilfried O Connell Casey Sykes David B 2012 Complete and Partial Responses of the TEMPI Syndrome to Bortezomib PDF N Engl J Med 367 8 778 780 doi 10 1056 NEJMc1205806 PMID 22913703 Sykes David B Schroyens W 2018 Complete Responses in the TEMPI Syndrome after Treatment with Daratumumab N Engl J Med 378 23 2240 2242 doi 10 1056 NEJMc1804415 PMID 29874534 S2CID 205064988 Kenderian S S Rosado F G Sykes D B Hoyer J D Lacy M Q 2015 Long term complete clinical and hematological responses of the TEMPI syndrome after autologous stem cell transplantation Leukemia 29 12 2414 2416 doi 10 1038 leu 2015 298 PMID 26500143 Bazari Hasan Attar Eyal C Dahl Douglas M Uppot Raul N Colvin Robert B 2010 Case Records of the Massachusetts General Hospital Case 23 2010 A 49 Year Old Man with Erythrocytosis Perinephric Fluid Collections and Renal Failure N Engl J Med 363 5 463 475 doi 10 1056 NEJMcpc1004086 PMID 20818867 External links editOffice of Rare Diseases Research US Orpha net EU Hematopia TEMPI Page Retrieved from https en wikipedia org w index php title TEMPI syndrome amp oldid 1218325448, wikipedia, wiki, book, books, library,
