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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

Carfilzomib-based triplet regimen a potential upfront treatment for high-risk MM

Pearl Toh, 22 Oct 2020

The combination therapy comprising carfilzomib, cyclophosphamide and dexamethasone (KCd) is effective, with a tolerable safety profile, in an Asian cohort with high-risk multiple myeloma (MM) — thus providing a more economical alternative as a potential upfront regimen in resource-limited settings, according to leading experts during a myeloma education webinar.

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13 Sep 2022

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ALL treatment: Current settings, future goals

Roshini Claire Anthony, 08 Jan 2021

Significant advancements in the treatment of acute lymphocytic leukaemia (ALL) in children have improved their outcomes. However, ALL treatment and eradication remain a major challenge in adults. In a recent webinar, two specialists from the US discussed current ALL treatment strategies and goals of future regimens.