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ET patient looking outside

ET is associated with a variety of signs and symptoms

ET patient looking outside

ET is associated with a variety of signs and symptoms

ET is characterized by1:
  • Erythromelalgia (burning or throbbing pain in the hands or feet)
  • Fatigue/weakness
  • Low-grade fever
  • Thrombus
  • Unexpected or exaggerated bleeding
  • Enlarged spleen
  • Fainting
  • Weight loss
  • Night sweats
  • Chest pain
Some ET symptoms are associated with compromised cerebral blood flow1:
  • Headache
  • Dizziness
  • Weakness or numbness on one side of the body
  • Slurred speech
  • Blurred or double vision

Symptom burden affects a substantial percentage of patients2

Patients assessed symptom burden according to the the Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS).2*

The vast majority of patients (87%) reported fatigue, and at least half reported early satiety, concentration problems, inactivity, abdominal discomfort, and night sweats.2

Image shows symptom burden according to the the Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS)

ET, essential thrombocythemia.

*Symptom assessment of 594 patients with ET. Incidence is a score of >0 on the MPN-SAF TSS. Symptom severity was rated on a 0 (absent/as good as it can be) to 10 (worst imaginable/as bad as it can be) scale. The MPN-SAF TSS has a possible range of 0 to 100, with 100 representing the highest level of symptom severity. Fatigue was independently measured by coadministration of the Brief Fatigue Inventory.2

ET may be difficult to diagnose

Diagnosing ET may be difficult because of its similarity to other myeloproliferative neoplasms (MPNs), and misdiagnosis can have an impact on patient outcomes.3,4

The International Working Group for MPN Research and Treatment (IWG-WRT) subjected 1104 cases of locally diagnosed ET to review by a central laboratory. The laboratory determined that 891 of the cases met the World Health Organization (WHO) criteria for ET, but 213 did not. They classified these cases as early/prefibrotic myelofibrosis (MF).4

Performing an accurate bone marrow biopsy for the differential diagnosis of ET from early/prefibrotic MF is important.

Management of ET is multi-faceted

The focus of managing ET is to5,6:

  • Avoid first occurrence and/or recurrence of thrombotic and bleeding complications
  • Minimize the risk of acute leukemia and post-polycythemia vera MF
  • Control systemic symptoms
  • Treat complications (thrombosis and hemorrhage)
  • Manage risk situation (eg, pregnancy, surgery)

References: 1. Leukemia & Lymphoma Society. Myeloproliferative neoplasms. https://www.lls.org/sites/default/files/National/USA/Pdf/Publications/MPNs_booklet_12_17_FINAL.pdf.  Accessed September 29, 2021. 2. Emanuel RM, Dueck AC, Geyer HL, et al. J Clin Oncol. 2012;30(33):4098-4103. 3. Tefferi A, Vardiman JW. Leukemia. 2008;22(1):14-22. 4. Barbui T, Thiele J, Passamonti F, et al. J Clin Oncol. 2011;29(23):3179-3184. 5. Beer PA, Green AR. Hematology Am Soc Hematol Educ Program. 2009:621-628. 6. Barbui T, Barosi G, Birgegard G, et al. J Clin Oncol. 2011;29(6):761-770.