Tempi syndrome
ORPHA: 284227
Overview
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
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with Tempi syndrome, sourced from HPO and Orphanet clinical annotations.
Abnormality of the kidneyTelangiectasiaIncreased hematocritIncreased circulating IgG concentrationHypoxemiaHemangiomaAscitesAbnormality of the pulmonary vasculatureTransudative pleural effusionPolycythemiaIntracranial hemorrhageVenous thrombosisFacial erythemaHeadache
Classification & Codes
Orphanet Code
ORPHA:284227Tempi syndrome
| Orphanet | ORPHA:284227 |
| Treatments | 0 drug(s) |
| Symptoms on record | 14 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO