The TEMPI syndrome

BLOOD SPOTLIGHT| APRIL 9, 2020

The TEMPI syndrome

David B. Sykes, Casey O』Connell, Wilfried Schroyens

Blood (2020) 135 (15): 1199–1203.

https://doi.org/10.1182/blood.2019004216

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.

Subjects:

Blood Spotlight, Clinical Trials and Observations, Lymphoid Neoplasia

Topics:

erythrocytosis, erythropoietin, monoclonal antibodies, monoclonal gammopathy of undetermined significance, paraproteinemias, shunt intrapulmonary, telangiectasis, signs and symptoms

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