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Primary myelofibrosis

Key sources
The following summarized guidelines for the evaluation and management of primary myelofibrosis are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2024; 2023), the European Leukemia Net (ELN 2018), the European Society of Medical Oncology (ESMO 2015), and the British Committee for Standards In Haematology (BCSH 2014; 2012).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: as per ELN 2018 guidelines, use the 2016 revised WHO criteria for diagnosis of PMF.
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2.Classification and risk stratification

Risk assessment: as per BSH 2023 guidelines, obtain prognostic evaluation using currently available validated scores in all patients with myelofibrosis.
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3.Diagnostic investigations

Initial evaluation: discuss all diagnoses in a specialist multidisciplinary meeting.
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  • Genetic testing

4.Diagnostic procedures

Bone marrow biopsy: as per BSH 2023 guidelines, perform a diagnostic bone marrow biopsy with molecular testing forJAK2 ,CALR , orMPL variants as appropriate in all patients with suspected myelofibrosis.
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  • Upper gastrointestinal endoscopy

5.Medical management

Watchful waiting: offer observation alone in patients with IPSS/DIPSS/DIPSS-plus low- or intermediate-1 risk myelofibrosis without significant symptoms, significant anemia (hemoglobin < 10 g/dL), splenomegaly (palpable spleen size > 10 cm), leukocytosis (WBC count > 25×10⁹/L), or marked thrombocytosis (platelet count > 1, 000×10⁹/L).
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  • Hydroxyurea

  • JAK inhibitors (indications)

  • JAK inhibitors (pretreatment evaluation and monitoring)

  • Conditioning regimens

  • Management of accelerated/blast phase

  • Management of anemia (general principles)

  • Management of anemia (ESAs)

  • Management of anemia (androgens)

  • Management of anemia (immunomodulatory drugs)

  • Management of anemia (corticosteroids)

  • Management of anemia (blood transfusion)

  • Management of anemia (iron chelation)

  • Management of iron overload

6.Therapeutic procedures

Splenic irradiation: as per BSH 2024 guidelines, consider offering splenic irradiation for symptomatic splenomegaly or splenic pain refractory to medical therapies in patients ineligible for splenectomy.
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  • Radiotherapy for extramedullary hematopoiesis

  • allo-SCT

7.Perioperative care

Perioperative management: consider controlling blood counts preoperatively to standard targets in high-risk patients undergoing surgery, when bleeding is a risk or thromboprophylaxis would normally be prescribed.
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8.Surgical interventions

Indications for splenectomy: as per ELN 2018 guidelines, offer splenectomy as a palliative treatment option for drug-refractory symptomatic splenomegaly, and consider offering when drug-induced anemia hampers the effective use of hydroxyurea or ruxolitinib.
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9.Specific circumstances

Pediatric patients: offer a conservative approach in most pediatric patients. Consider offering a trial of corticosteroids once acute megakaryoblastic leukemia and vitamin D deficiency have been excluded.
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  • Breastfeeding patients

  • Patients on hormonal therapy

  • Patients with leukemic transformation

10.Follow-up and surveillance

Assessment of treatment response: consider using the Myeloproliferative Neoplasm Symptom Assessment Form for objective monitoring of symptoms.
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