Orphanet: TEMPI syndrome

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TEMPI syndrome

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • Telangiectasia-erythrocytosis-monoclonal gammopathy-perinephric-fluid collections-intrapulmonary shunting syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: -
  • Age of onset: Adult
  • ICD-10: -
  • OMIM: -
  • UMLS: C3854394
  • MeSH: -
  • GARD: 10962
  • MedDRA: -
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