Background: The TEMPI syndrome was recently described in 2011, and is characterized by the constellation of five\nhallmarks: Telangiectasias, Erythrocytosis and elevated Erythropoietin, Monoclonal gammopathy, Perinephric fluids\ncollections, and Intrapulmonary shunting. The underlying pathophysiology is unknown, though it has been postulated\nthat the monoclonal gammopathy may play a causal role.\nCase presentation: A 37-year-old non-smoking male presented to our institution with a fever and the sensation of\nfullness in the right flank. His exam was notable for telangiectasias, clubbing of the fingernails, plethora, and a palpable\nbulge in the right flank. Renal ultrasound demonstrated bilateral perinephric fluid collections. Laboratory evaluation\nrevealed erythrocytosis with low serum erythropoietin, and testing for the JAK2V617F mutation was positive, confirming\na diagnosis of polycythemia vera. Though his room air saturation was normal at rest, it decreased dramatically with\nexercise, felt to be secondary to microscopic intrapulmonary shunting.\nThe patient�s presentation is very similar to that of the TEMPI syndrome, a very rare syndrome of which there have\nbeen six published cases. In contrast to the TEMPI syndrome where the erythrocytosis is driven by highly elevated\nserum erythropoietin, our patient was found to have polycythemia vera. Also in contrast to the other patients with\nTEMPI syndrome, our patient did not have an identifiable monoclonal gammopathy.\nOur patient responded to treatment with hydroxyurea. His erythrocytosis, perinephric fluid collections, and\ntelangiectasias resolved over the course of six months. The intrapulmonary shunting has continued to gradually\nimprove with treatment, suggesting that this is an entirely reversible process.\nConclusion: Our case is the first to describe the combination of polycythemia vera, telangiectasias, perinephric fluid\ncollections, and intrapulmonary shunting. The presentation is highly similar to the previously described TEMPI\nsyndrome, though calls into question the potential importance of the monoclonal gammopathy. Our patient\ndemonstrated a response to treatment with hydroxyurea, while patients with the TEMPI syndrome have shown\nresponses to plasma-cell directed therapies such as bortezomib.
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