The TEMPI syndrome is a rare and acquired disorder characterized by five salient features: (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 that 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.

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