Objectives
  • To be able to diagnose myeloproliferative neoplasms (MPNs), to discriminate them from reactive blood disorders and to perform specialist assessment which includes interpretation of molecular diagnostic data, treatment according to patient and disease-related risk groups and counselling of patients

Awareness
  • Awareness of MPNs as a differential diagnosis in patients with altered blood counts and/or splenomegaly; frequent subtypes: polycythaemia vera (PV), essential thrombocythaemia (ET), primary myelofibrosis (PMF)

  • Awareness of rare MPN varieties such as mastocytosis, chronic eosinophilic leukaemia, diseases with abnormalities of platelet-derived growth factor receptor A/B (PDGFRA/B), fibroblast growth factor receptor 1 (FGFR1)

  • Awareness of the availability of different diagnostic procedures

  • Recognition of the existence of different prognostic factors

  • Awareness of the variety of different treatment options

Knowledge
  • Understanding of the implications of the different subtypes

  • Familiarity with diagnostic criteria for main subtypes PV, ET, PMF and diagnostic algorithms

  • Familiarity with the risk assessment work-up of prognostic factors, specifically risks of thrombosis and bleeding, considering pre-existing conditions (comorbidities, previous risk factors), risk-reduction options

  • Familiarity with possible progression scenarios—leukaemic transformation/secondary fibrosis

  • Familiarity with treatment options, supportive care, symptomatic treatment, role of antithrombotic agents, indications, risks and value for cytoreductive therapies, interferon, splenic irradiation, splenectomy, new targeted treatment approaches, role of allogeneic transplant, palliation

  • Understanding of treatment personalisation opportunities from molecular findings

  • Understanding how to discriminate complications derived from disease progression from those treatment-related

Skills
  • Ability to contribute actively to the work-up of patients with suspected MPNs, that includes performing bone marrow aspiration and biopsies and ordering the necessary work-up of the material collected and interpretation of data

  • Ability to contribute actively in case presentations and to discuss critically treatment options

  • Ability to perform history and physical examination in MPN patients of different subtypes

  • Ability to correctly assess the significance of Janus kinase 2 (JAK2) mutations, of smoking cessation and of phlebotomy for PV as an example

  • Ability to contribute to discussions on general management strategies

  • Ability to recognise disease-specific conditions that are important for considering when to start and to stop treatment, which treatment option to choose and when to switch

  • Ability to recognise patient-specific conditions/comorbidities that are important to choose between treatment options

  • Ability to determine therapy according to pathology findings and molecular marker status

  • Ability to consider MPNs as chronic disorders with implications for long-term follow-up

  • Ability to manage side effects of treatment