Expert Insights

Part 5: Symptom Burden

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Narrator: Clinical characteristics of advanced PV may include hematocrit greater than or equal to 45% plus one additional risk factor despite treatment with hydroxyurea and phlebotomy.

Specifically, clinical characteristics of advanced PV may include hematocrit greater than or equal to 45% plus: elevated white blood cell count greater than 11x109 per liter or burdensome disease-related symptoms (such as fatigue, early satiety, inactivity, concentration problems, and itching).

Dr Shammo: I think the symptom burden of patients who have PV have been largely underrepresented and underappreciated.

Narrator: A prospective study of 1334 patients with PV was conducted by Geyer, et al. to assess baseline symptoms with certain disease features (eg, known hydroxyurea use… Assessment of myeloproliferative neoplasm (MPN) symptoms was performed by using the MPN-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN-10). Patients were assigned to symptom severity quartiles based on TSS score.

Dr Shammo: And as you see from the Geyer study, patients who had been treated with hydroxyurea or are currently taking it, continue to have substantial symptom burden.

That's somewhat surprising because you would think that if somebody's receiving cytoreductive therapy, they should actually have improved symptomatology if the disease is being under control.

Narrator: Actively monitor for hematocrit plus in your patients with polycythemia vera.

Image of Jamile M Shammo, MD, FASCP, FACP
Jamile M Shammo, MD, FASCP, FACP Associate Professor Rush University Medical Center | Chicago, IL
As principal investigator of clinical trials in her area of expertise, Dr Shammo is heavily involved in education, research, and administrative activities in the Division of Hematology/Oncology. She is recognized nationally for her expertise in bone marrow failure syndromes/paroxysmal nocturnal hemoglobinuria (PNH) and was chosen to serve as a national coordinator for the US PNH registry.