tempi syndrome
MONDO:0017286Mondo
Findings
No curated finding names tempi syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0017286), read 2026-09-29. CC BY 4.0.
Features
14 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormality of the kidneyHPOHP:0000077
- Obligate (100% of cases)
- HypoxemiaHPOHP:0012418
- Obligate (100% of cases)
- Increased circulating IgG concentrationHPOHP:0003237
- Obligate (100% of cases)
- Increased hematocritHPOHP:0001899
- Obligate (100% of cases)
- TelangiectasiaHPOHP:0001009
- Obligate (100% of cases)
- Abnormality of the pulmonary vasculatureHPOHP:0004930
- Very frequent (80% to 99% of cases)
- AscitesHPOHP:0001541
- Very frequent (80% to 99% of cases)
- HemangiomaHPOHP:0001028
- Very frequent (80% to 99% of cases)
- Transudative pleural effusionHPOHP:0011920
- Very frequent (80% to 99% of cases)
- Intracranial hemorrhageHPOHP:0002170
- Frequent (30% to 79% of cases)
- PolycythemiaHPOHP:0001901
- Frequent (30% to 79% of cases)
- Venous thrombosisHPOHP:0004936
- Frequent (30% to 79% of cases)
Show the remaining 2
- Facial erythemaHPOHP:0001041
- Occasional (5% to 29% of cases)
- HeadacheHPOHP:0002315
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: tempi syndrome
- Also called
- telangiectasia-erythrocytosis-monoclonal gammopathy-perinephric-fluid collections-intrapulmonary shunting syndrome