TEMPI 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.

TEMPI syndrome
Other names	Telangiectasia-erythrocytosis-monoclonal gammopathy-perinephric-fluid collections-intrapulmonary shunting syndrome

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. Abnormal plasma-cell clone and monoclonal gammopathy are suggested to be triggers of the disease.^[2]

Diagnosis

The diagnosis is based on the five characteristics described above.^[2]

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]