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INGRAIN-MF
Incorporating genetics, risk and associated burden into MF management

MODULE 3: A personalised approach to predicting prognosis in myelofibrosis

Having completed this module, you should now have a better appreciation of:

  • clinical and demographic risk factors, and their influence on survival
  • cytogenetic and genetic risk factors, and their influence on survival
  • different prognostic scoring systems and how they compare
  • the use and implications of risk stratification

Retest your knowledge below.

Post-module multiple-choice questions

When your answers have been submitted, you will be able to review the correct answers and receive your final score.

Correct answer is highlighted.

Answer: The IPSS, on which some subsequent algorithms were based, is composed of age >65 years (1 point), constitutional symptoms (1 point), Hb <10 g/dL (1 point), leukocyte count >25 x 109/L (1 point) and circulating blastocytes ≥1% (1 point). Splenomegaly is not included.1

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Answer: The GIPSS (Genetically Inspired Prognostic Scoring System) includes karyotype, absence of the CALR driver mutation and the presence of a high molecular risk non-driver mutation.2

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Answer: The Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM) was developed using only patients with secondary MF, and scores clinical and genetic factors to predict survival.3

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Answer: Due to the possible complications associated with allogeneic stem cell transplant, it is suggested that patients whose median survival is less than 5 years, and who are otherwise eligible, be considered for transplantation.4

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Answer: NICE reimbursement for ruxolitinib is available only for patients with intermediate-2 or high risk disease.5

CALR: calreticulin; ET: essential thrombocythaemia; NICE: National Institute for Health and Care Excellence; PV: polycythaemia vera

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Adverse events should be reported. Reporting forms and information can be found at www.mhra.gov.uk/yellowcard.
Adverse events should also be reported to Novartis online through the pharmacovigilance intake (PVI) tool at www.novartis.com/report or alternatively email medinfo.uk@novartis.com or call 01276 698370

References
  1. Cervantes F, Dupriez B et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood 2009;113(13):2895-2901
  2. Tefferi A, Guglielmelli P et al. GIPSS: Genetically Inspired Prognostic Scoring System for primary myelofibrosis. Leukemia 2018;32(7):1631-1642
  3. Passamonti F, Giorgino T et al. A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis. Leukemia 2017;31(12):2726-2731
  4. Barbui T, Barosi G et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol 2011;29(6):761-770
  5. National Institute for Health and Care Excellence (NICE). Technology appraisal guidance 386. Ruxolitinib for treating disease-related splenomegaly or symptoms in adults with myelofibrosis, March 2016 (last reviewed July 2019). Available at: nice.org.uk. Accessed August 2025

© NICE 2016 Ruxolitinib for treating disease-related splenomegaly or symptoms in adults with myelofibrosis. Available from www.nice.org.uk/guidance/ta386. All rights reserved. Subject to Notice of rights.

NICE guidance is prepared for the National Health Service in England. All NICE guidance is subject to regular review and may be updated or withdrawn. NICE accepts no responsibility for the use of its content in this product/ publication.