The TEMPI syndrome
David B. Sykes, Casey L. O’Connell, Wilfried Schroyens
Abstract
The TEMPI syndrome is a rare and acquired disorder characterized by 5 salient features, which compose its name: (1) telangiectasias; (2) elevated erythropoietin and erythrocytosis; (3) monoclonal gammopathy; (4) perinephric fluid collections; and (5) intrapulmonary shunting. Complete resolution of symptoms following treatment with plasma cell-directed therapy supports the hypothesis that the monoclonal antibody is causal and pathogenic. Understanding the basis of the TEMPI syndrome will depend on the identification of additional patients and a coordinated international effort.
Topics & Concepts
Monoclonal gammopathyMedicineMonoclonalErythropoietinInternal medicinePathologyMonoclonal antibodyGastroenterologyImmunologyAntibodyVascular Malformations and HemangiomasVascular Malformations Diagnosis and TreatmentCoagulation, Bradykinin, Polyphosphates, and Angioedema