myelofibrosis
MYELOFIBROSIS
Treatment Guideline Chart

A clonal myeloproliferative stem cell disorder characterized by reactive bone marrow fibrosis, extramedullary hematopoiesis, and abnormal cytokine expression leading to systemic symptoms.
It belongs to a group of heterogeneous disorders of the hematopoietic system which is collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN).
May present either as a de novo disorder [primary myelofibrosis (PMF)] or can develop from the transformation of polycythemia vera (PV) and essential thrombocythemia (ET).

Choice of treatment for patients with myelofibrosus is based on the risk score and presence of symptoms

Myelofibrosis Signs and Symptoms

Definition

  • Clonal myeloproliferative stem cell disorder characterized by reactive bone marrow fibrosis, extramedullary hematopoiesis, and abnormal cytokine expression leading to systemic symptoms 
  • Present either as a de novo disorder [primary myelofibrosis (PMF)] or can develop from the transformation of polycythemia vera (PV) and essential thrombocythemia (ET) 
  • PMF is characterized by clonal proliferation of early hematopoietic stem cells 
    • Often accompanied by Janus kinase 2 (JAK2), calreticulin (CALR) or myeloproliferative leukemia (MPL) gene mutations 
    • Sub-classified into prefibrotic or early stage PMF and overt fibrotic-stage PMF 
    • Occurs mainly in middle aged and older adults 
      • Median age of onset is 67 years 
    • Most common presenting symptom is fatigue and other symptoms include enlarged spleen, weight loss, low grade fever, night sweats, bone pain

Introduction

  • Belongs to a group of heterogeneous disorders of the hematopoietic system which is collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN)

Signs and Symptoms

  • Constitutional symptoms include fatigue, fever, weight loss and night sweats 
  • Severe anemia, bone pain, splenic infarct, pruritus, thrombosis, bleeding, cachexia 
  • Approximately 30% are asymptomatic
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