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Case report: a 37-year-old male with telangiectasias, polycythemia vera, perinephric fluid collections, and intrapulmonary shunting

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2014

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Springer Nature
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Khan, Javed, and David B Sykes. 2014. “Case Report: a 37-Year-Old Male with Telangiectasias, Polycythemia Vera, Perinephric Fluid Collections, and Intrapulmonary Shunting.” BMC Hematology 14 (1) (July 22). doi:10.1186/2052-1839-14-11.

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Abstract

The TEMPI syndrome was recently described in 2011, and is characterized by the constellation of five hallmarks: Telangiectasias, Erythrocytosis and elevated Erythropoietin, Monoclonal gammopathy, Perinephric fluids collections, and Intrapulmonary shunting. The underlying pathophysiology is unknown, though it has been postulated that the monoclonal gammopathy may play a causal role.

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Erythrocytosis,, Erythropoietin, Monoclonal gammopathy, Perinephric fluid, Polycythemia, TEMPI

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