TEMPI syndrome
ORPHA:284227· ICD-10 D47.2
Definition
TEMPI syndrome is a rare multi-systemic disease characterized by the presence of Telangiectasias, Erythrocytosis with elevated erythropoietin levels, Monoclonal gammopathy, Perinephric-fluid collections, and Intrapulmonary shunting.
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adult