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Primary myelofibrosis

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Updated 2023 BSH guidelines for the management of primary myelofibrosis.

Guidelines

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 American Society for Transplantation and Cellular Therapy & Cell Therapy Transplant Canada (ASTCT 2024), the British Society for Haematology (BSH 2024), the European Leukemia Net (ELN 2018), the European Society of Medical Oncology (ESMO 2015), and the ...
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Screening and diagnosis

Diagnostic criteria: as per ELN 2018 guidelines, use the 2016 revised WHO criteria for diagnosis of PMF.
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WHO criteria for overt primary myelofibrosis
Major criteria
Presence of megakaryocytic proliferation and atypia, accompanied by either reticulin and/or collagen fibrosis grades 2 or 3
Not meeting the WHO criteria for essential thrombocythemia, polycythemia vera, BCR-ABL11 chronic myeloid leukemia, myelodysplastic syndromes, or other myeloid neoplasms
Presence of JAK2, CALR, or MPL mutation or in the absence of these mutations, presence of another clonal marker, or absence of reactive myelofibrosis
Minor criteria (presence confirmed in 2 consecutive determinations)
Palpable splenomegaly
Anemia not attributed to a comorbid condition
WBC count ≥ 11×10⁹/L
LDH increased to above upper normal limit of institutional reference range
Leukoerythroblastosis
Diagnostic criteria are not met
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Classification and risk stratification

Risk assessment: as per BSH 2024 guidelines, obtain prognostic evaluation using currently available validated scores in all patients with myelofibrosis.
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Diagnostic investigations

Initial evaluation: as per BSH 2024 guidelines, discuss all diagnoses in a specialist multidisciplinary meeting.
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  • Genetic testing

Diagnostic procedures

Bone marrow biopsy: as per BSH 2024 guidelines, perform a diagnostic bone marrow biopsy with molecular testing for JAK2, CALR, or MPL variants as appropriate in all patients with suspected myelofibrosis.
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  • Upper gastrointestinal endoscopy

Medical management

Watchful waiting: as per ELN 2018 guidelines, 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

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

  • Management of donor-specific anti-HLA antibodies (prevention)

  • Management of donor-specific anti-HLA antibodies (testing)

  • Management of donor-specific anti-HLA antibodies (desensitization therapy)

  • Management of donor-specific anti-HLA antibodies (follow-up)

  • Management of non-donor-specific anti-HLA antibodies

Perioperative care

Perioperative management: as per ESMO 2015 guidelines, 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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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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Specific circumstances

Pediatric patients: as per BCSH 2012 guidelines, 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

Follow-up and surveillance

Assessment of treatment response: as per BCSH 2014 guidelines, consider using the Myeloproliferative Neoplasm Symptom Assessment Form for objective monitoring of symptoms.
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