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Expert Breaks Down Diagnosis and Treatment of Classical MPNs

Angelica Welch
Published: Thursday, Oct 18, 2018

Vivian G. Oehler, MD

Vivian G. Oehler, MD

Myeloproliferative neoplasms (MPNs) are a spectrum of hematologic diseases in which factors of diagnosis and treatment vary, according to Vivian G. Oehler, MD.

State of the Science Summit™ on Hematologic Malignancies, Oehler, an associate member of the Fred Hutchinson Cancer Research Center, discussed the diagnosis, risk prognostication and therapeutic strategies for polycythemia vera (PV), essential thrombocytopenia (ET), and myelofibrosis (MF).

Polycythemia Vera

In 2017, the World Health Organization (WHO) defined PV with updated criteria. Hemoglobin has been lowered to >16.5g/dL in men and >16 g/dL in women, bone marrow with trilineage growth, and the presence of a JAK2 mutation.

Data from MPD-RC 112 (NCT01259856) is anticipated to be released soon, hinted Oehler. This recently completed phase III trial evaluated frontline peg-IFN-α2a with hydroxyurea in patients with high-risk PV and ET.

Essential Thrombocytopenia

The major criteria for ET is a platelet count of great than or equal to 450 x 109/L; proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytic with hyperlobulated nuclei, no significant increase or left shift in neutrophil granulopoiesis or erythropoiesis and very rare minor increase in reticulin fibers; not meeting WHO criteria for chronic myeloid leukemia, PV, pre-fibrotic primary MF (prePMF), myelodysplastic syndrome, or other myeloid neoplasms; and presence of a JAK2, CALR, or MPL mutation. Minor criteria are the presence of a clinical marker or absence of evidence for relative thrombocytosis.
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