TEMPI syndrome

Common Name(s)

TEMPI syndrome, Telangiectasia - Erythrocytosis - Monoclonal Gammopathy - Perinephric-Fluid Collections - Intrapulmonary Shunting

TEMPI syndrome is a newly discovered, multisystem condition named for 5 characteristics that affected individuals have: Telangiectasias, Erythrocytosis with elevated erythropoietin level, Monoclonal gammopathy, Perinephric-fluid collections (fluid around the kidney), and Intrapulmonary shunting (when a region of the lungs is supplied with blood but with little or no ventilation).  Signs and symptoms of TEMPI syndrome have appeared in mid-adulthood in all known affected individuals. The telangiectasias develop mostly on the face, trunk and arms. The intrapulmonary shunt eventually leads to hypoxia (inadequate oxygen supply). Blood clots and bleeding in the brain have also been reported in some affected individuals. The cause of TEMPI syndrome is currently unknown. Treatment has reportedly been completely or partially successful with the medication bortezomib.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "TEMPI syndrome" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "TEMPI syndrome" returned 4 free, full-text research articles on human participants. First 3 results:

Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist.
 

Author(s): Flavia G Rosado, Jennifer L Oliveira, Aliya R Sohani, Wilfried Schroyens, David B Sykes, Saad S Kenderian, Martha Q Lacy, Robert A Kyle, James D Hoyer

Journal: Mod. Pathol.. 2015 Mar;28(3):367-72.

 

TEMPI syndrome (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) is a recently described syndrome that, owing to erythrocytosis, may be confused with polycythemia vera. It is best ...

Last Updated: 27 Feb 2015

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Complete and partial responses of the TEMPI syndrome to bortezomib.
 

Author(s): Wilfried Schroyens, Casey O'Connell, David B Sykes

Journal: N. Engl. J. Med.. 2012 Aug;367(8):778-80.

 

Last Updated: 23 Aug 2012

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Bortezomib to treat the TEMPI syndrome.
 

Author(s): Mary Kwok, Neha Korde, Ola Landgren

Journal: N. Engl. J. Med.. 2012 May;366(19):1843-5.

 

Last Updated: 10 May 2012

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "TEMPI syndrome" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

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