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MYELOFIBROSIS

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

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

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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Immunotherapy sparks hope for paediatric ALL

Audrey Abella, 14 Oct 2020

Despite a 90-percent cure rate after first treatment for children with acute lymphoblastic leukaemia (ALL), approximately 10–15 percent of patients with paediatric ALL will experience relapse. [Expert Rev Anticancer Ther 2017;17:725-736] A recent webinar on the current landscape of ALL highlighted the potential of immunotherapy for paediatric patients with relapsed or refractory ALL, thus providing hope for this high-risk patient group.