Advances in the Treatment of Myeloproliferative Disorders

Andrew Schorr:

All right. Let's understand. When we talk about a myeloproliferative disorder, first of all, what was Marty's specific diagnosis? How common is this? What is the range of these problems? What's going on, basically?

Dr. Verstovsek:

When we talk about myeloproliferative diseases we in principle talk about the so called Philadelphia classic chromosome negative Myeloproliferative diseases, which are three. One is called essential thrombocythemia, or ET, where the major problem is high platelets. It's a pretty benign disease, and patients live long life by controlling the platelet number. Polycythemia vera is the second one where patients have a high number of red blood cells, white cells and platelets in the blood, and again usually patients live a prolonged period of time, decades, by controlling the numbers and don't suffer much from the disease.

However primary myelofibrosis, or MF, is the one that Marty had, and this is the one where the bone marrow reacts to the presence of the disease by producing fibers, and the bone marrow very quickly becomes fibrotic like a scar tissue in the bone marrow limiting the number of cells, so the patients instead of having too much cells present like Martin had, with anemia. Many also suffer from cachexia, meaning losing weight because the disease takes a toll on the body, the weakness. The enlargement of the spleen and liver can happen because these organs take over production of blood cells from the bone marrow. And this disease, because of all the signs and symptoms, significantly shortens the life expectancy to several years. Usually people say about five to seven years on average, but it's been from few years to more than a decade.

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Page last updated on December 18, 2013