TEMPI syndrome | |
---|---|
Other names | 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.
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]
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]
The diagnosis is based on the five characteristics described above. [2]
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]
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]
TEMPI syndrome | |
---|---|
Other names | 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.
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]
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]
The diagnosis is based on the five characteristics described above. [2]
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]
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]