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Blood. 2020 Feb 27. pii: blood.2019004216. doi: 10.1182/blood.2019004216. [Epub ahead of print]

The TEMPI Syndrome.

Author information

1
Massachusetts General Hosptial, Boston, Massachusetts, United States.
2
University of Southern California, Los Angeles, California, United States.
3
University of Antwerp, Edegem, Belgium.

Abstract

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.

PMID:
32108223
DOI:
10.1182/blood.2019004216

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