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The TEMPI Syndrome

Erythropoietin & Erythrocytosis
Monoclonal gammopathy (MGUS)
Perinephric fluid
Intrapulmonary shunting


The TEMPI Syndrome is a very rare orphan disease. Affected individuals typically display the five hallmarks from which the acronym is derived: telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting. All patients have high erythropoietin levels and an MGUS (also called a Monoclonal Gammopathy of Undetermined Significance), but not all patients have perinephric fluid or intrapulmonary shunting early in the course of the disease. 

As of March 2024, we have identified a total of 41 people with TEMPI syndrome in the world.

Click on the Google map --> to see where the patients are located.

Additional Information can be found in the links below!


2018 International TEMPI Symposium

On November 30, 2018 the first TEMPI symposium was held as part of the American Society of Hematology annual convention in San Diego, California. We brought together an international group of physicians to discuss the pathophysiology of this unusual condition and to put together a plan for sample collection and research to help best define treatment options for our patients.  

Do you think that you or your patient might have TEMPI Syndrome?

Please download and complete our TEMPI Syndrome worksheet.

Collecting and banking a bone marrow sample? 

Please feel free to use our protocol for banking bone marrow mononuclear cells.

Shipping bone marrow internationally?


Consider the PAXgene bone marrow RNA tubes and kit (Qiagen)

Would you like to donate to TEMPI Syndrome research?

It can be challenging funding research for a disease where only 41 people in the world are affected.

Please consider making a donation!

Specify: "TEMPI Syndrome, Dr. David Sykes Research Fund."


Last updated: March 12, 2024

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