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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Dr. Wong Seng Weng, Dr. Lee Guek Eng, 19 Mar 2021

In Singapore, advanced breast cancer (aBC) is the leading cause of cancer death in women 30–49 years of age. For premenopausal women diagnosed with hormone receptor (HR)-positive, HER2-negative BC, cyclin-dependent kinase 4 and 6 (CDK4/6) inhibitors provide new hope. Currently, ribociclib is the only CDK4/6 inhibitor studied in the first-line setting for premenopausal women with HR+/HER2- aBC, in the phase III MONALEESA-7 trial. Two experts, Dr Wong Seng Weng, Medical Director & Consultant Medical Oncologist at The Cancer Centre, Paragon Medical & Mount Elizabeth Novena Specialist Centre, and Dr Lee Guek Eng, Senior Consultant & Specialist in Medical Oncology at Icon Cancer Centre and Program Director of the Young Women Cancer Program, weigh in with their views on managing premenopausal women with aBC.

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.

New treatment option now available for patients with HR+/HER2- aBC/MBC, PIK3CA mutations

31 Mar 2021

At the recent webinar entitled Pik Me Up: Mutation Matters, Consultant Medical Oncologist Dr Senthil Rajappa discussed the role of PI3Kα-specific inhibitor alpelisib (TREZILENT^®, Novartis) in managing patients with HR+/HER2- aBC/MBC and PIK3CA mutations while Senior Consultant Pathologist Professor Dr Pathmanathan Rajadurai explained the process of detecting PIK3CA mutations in patients.