TEMPI syndrome

Also known as: Telangiectasias, elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting

Rare syndrome with erythrocytosis and monoclonal gammopathy.

Medical specialty: Hematology
Subspecialty: Hematology / Plasma cell disorders
Sex & age distribution: Male: affected; Female: affected; Children: no
Gene(s) involved: None recorded

Etiology & mechanism

Unknown, dysregulated erythropoietin.

Gene(s) involved (0)

No gene recorded.

Symptoms & signs (6)

dyspneaerythrocytosisintrapulmonary shuntingmonoclonal gammopathyperinephric fluidtelangiectasias

SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.