TEMPI syndrome explained

TEMPI syndrome
Synonyms:	Telangiectasia-erythrocytosis-monoclonal gammopathy-perinephric-fluid collections-intrapulmonary shunting syndrome

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.

Signs and symptoms

TEMPI	Symptom
T	Telangiectasias
E	Elevated Erythropoietin and Erythrocytosis
M	Monoclonal gammopathy
P	Perinephric fluid collections
I	Intrapulmonary shunting

The patients were all diagnosed at middle age. A monoclonal gammopathy was implicated in all patients tested.^[1]

Cause

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]

Diagnosis

The diagnosis is based on the five characteristics described above.

Treatment

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]

History

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]

References

Sykes. David B. . Schroyens . Wilfried . O'Connell . Casey . TEMPI Syndrome – A Novel Multisystem Disease . . 5 . 475–477 . 2011 . 10.1056/NEJMc1106670 . 21812700 . 365. 35990145 . free .
Sykes. David B.. O'Connell. Casey. Schroyens. Wilfried. 2020-04-09. The TEMPI syndrome. Blood. 135. 15. 1199–1203. 10.1182/blood.2019004216. 1528-0020. 32108223. free.
Schroyens . Wilfried . O'Connell . Casey . Sykes. David B. . Complete and Partial Responses of the TEMPI Syndrome to Bortezomib . . 367 . 8 . 778–780 . 2012 . 10.1056/NEJMc1205806. 22913703 .
Sykes . David B. . Schroyens . W. . Complete Responses in the TEMPI Syndrome after Treatment with Daratumumab . . 378 . 23 . 2240–2242 . 2018 . 10.1056/NEJMc1804415. 29874534 . 205064988 .
Kenderian . S.S.. . Rosado . F.G . Sykes . D.B. . Hoyer . J.D. . Lacy . M.Q. . Long-term complete clinical and hematological responses of the TEMPI syndrome after autologous stem cell transplantation . . 29 . 12 . 2414–2416 . 2015 . 10.1038/leu.2015.298. 26500143 . free .
Bazari . Hasan . Attar . Eyal C.. Dahl . Douglas M. . Uppot . Raul N. . Colvin . Robert B. . Case Records of the Massachusetts General Hospital. Case 23-2010: A 49-Year-Old Man with Erythrocytosis, Perinephric Fluid Collections, and Renal Failure . . 363 . 5 . 463–475 . 2010 . 10.1056/NEJMcpc1004086. 20818867 .