Leukemia 2021 Apr 28
Are polycythemia vera, essential thrombocytosis, and primary myelofibrosis 1, 2, or 3 diseases?
Related Questions
In patients with essential thrombocythemia (ET) receiving hydroxyurea for cytoreduction who develop anemia — whether hydroxyurea-induced or from a concurrent etiology such as CKD — would you use ESAs?
This is an important question. Patients with ET should not have anemia related to the ET itself. Thus, assessing the etiology of the anemia is very important. If the anemia is from hydroxyurea, the first step is to lower the dose of the hydroxyurea. Controlling platelets should not come at the expen...
For low/intermediate essential thrombocythemia not on cytoreduction, would you temporarily cytoreduce in anticipation of surgery?
First, I am going to answer this question with a question. What is low/intermediate essential thrombocytosis (ET)? I ask this question because we are living in the 21st century in the midst of the genomic era, and it is far past the time that we should be dwelling on phenotype instead of employing p...
How do you treat triple positive essential thrombocythemia with evidence of fibrosis on bone marrow biopsy, but does not meet diagnostic criteria for myelofibrosis?
My first thought upon encountering this question was that there must have been a typographical error and the writer actually meant “negative”, not positive, because “triple positive” essential thrombocythemia (ET) is an oxymoron. MPN driver mutations are not mutually exclusive with respect to their ...
Under which circumstances would you prefer ropeginterferon over hydroxyurea for patients with Polycythemia Vera?
The approval of ropeginterferon (Besremi) for the treatment of polycythemia vera (PV) without any restrictions was a welcome event for PV patients and their physicians. First, because this drug selectively targets the JAK2-mutated hematopoietic stem cell (HSC) responsible for PV and can produce comp...
In someone with an asymptomatic JAK2 V617 mutated and BCR/ABL negative MPN with a steadily rising WBC, when do you decide to start cytoreductive therapy and which agent(s) do you recommend?
First, let's abandon the phase "BCR/ABL-negative MPN". This is an oxymoron because BCR/ABL can be expressed in a JAK2 driver mutation-positive MPN. In fact, both BCR/ABL and JAK2 mutations can be expressed in normal individuals at very low levels without causing disease. Both mutations can also be p...
How do you differentiate between JAK2 positive ET and PV when peripheral blood shows erythrocytosis and thrombocytosis?
The answer to this question is very simple. Before the discovery of erythropoietin and accurate assays for it, and before the discovery of JAK2 driver mutations, the diagnosis of polycythemia vera was clinically-based. The Polycythemia Vera Study Group (PVSG) did an extensive epidemiologic study and...