Myelofibrosis

Overview

Myelofibrosis, an uncommon form of bone marrow cancer, disrupts the normal production of blood cells, leading to extensive scarring in the bone marrow. This scarring results in severe anemia, weakness, and fatigue, along with a reduced number of platelets, increasing the risk of bleeding. Additionally, myelofibrosis often manifests with an enlarged spleen. Classified as a chronic leukemia, myelofibrosis is part of a group of disorders known as myeloproliferative disorders, occurring either independently (primary myelofibrosis) or as a progression from another bone marrow disorder (secondary myelofibrosis).

While some individuals with myelofibrosis may remain asymptomatic and not require immediate treatment, others with more severe presentations may necessitate aggressive interventions. Treatment options for myelofibrosis, aimed at alleviating symptoms, can vary widely.

Symptoms

Myelofibrosis typically progresses slowly, often without noticeable symptoms during its early stages. However, as the disorder disrupts normal blood cell production, various signs and symptoms may manifest, including:

• Fatigue, weakness, or shortness of breath, often due to anemia.
• Pain or a feeling of fullness below the ribs on the left side, stemming from an enlarged spleen.
• Easy bruising or bleeding tendencies.
• Experiencing excessive sweating during sleep (night sweats).
• Fever.
• Bone pain.

It’s essential to consult a healthcare professional if you experience persistent signs or symptoms that cause concern.

Causes

Myelofibrosis is characterized by genetic mutations in bone marrow stem cells, which disrupt the normal process of blood cell production. The exact cause of these mutations remains unclear. As the mutated stem cells replicate, they pass on the mutations, leading to an imbalance in blood cell types, with a decrease in red blood cells causing anemia, an increase in white blood cells, and variable platelet levels. This results in the scarring of the typically spongy bone marrow. Common mutations include JAK2, CALR, and MPL, while some cases lack identifiable gene mutations. Identifying these mutations is crucial for prognosis and treatment decisions.

Risk factors

While the origin of myelofibrosis frequently remains unclear, certain factors are recognized for elevating your risk:

• Age: While myelofibrosis can impact individuals of any age, it is more frequently identified in those aged 50 and older. 
• Another blood cell disorder: A minority of individuals with myelofibrosis experience it as a complication arising from essential thrombocythemia or polycythemia vera. 
• Exposure to certain chemicals: Myelofibrosis has been associated with exposure to industrial chemicals like toluene and benzene. 
• Exposure to radiation: Individuals subjected to elevated levels of radiation face an augmented risk of developing myelofibrosis.