Published on October 14, 2015
In this Ask the Expert segment, Harriett writes that she was recently diagnosed with pulmonary hypertension and wants to know if the condition is related to her polycythemia vera. Dr. Kim-Hien Dao from the OHSU Knight Cancer Institute responds.
Transcript | Is There a Connection Between PV and Pulmonary Hypertension?
Here's a question we got from Harriet. She writes, “From 1986 until 2007, I had PV, polycythemia vera, and then it progressed to myelofibrosis. Last week I was diagnosed with pulmonary hypertension. Is there any connection between both diseases?“
Yes. So long-standing PV patients can develop pulmonary hypertension. First things first, though, I always work up the cause for the pulmonary hypertension. And so the common things—common causes of pulmonary hypertension include emphysema, COPD, obstructive sleep apnea, and also cardiovascular reasons for having pulmonary hypertension. So I make sure that those things are also worked up before I attribute it to long-standing polycythemia vera.
There is a condition called extramedullary hematopoiesis that can occur in the pulmonary system that can increase the blood pressure in that system, and so that would be the link to long-standing pulmonary—polycythemia vera or myelofibrosis development.
All right. But someone living with PV shouldn't routinely worry about pulmonary hypertension.
Correct. Yes. And so if that comes up in a setting of myelofibrosis, I certainly would, you know, do the regular test to rule out other common causes for pulmonary hypertension and then look at the lung tissue to a CAT scan to see if there is any extramedullary hematopoiesis.