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MYELOFIBRISIS

Myelofibrosis is a type of blood cancer that impairs the functioning of the bone marrow. The bone marrow produces stem cells that are responsible for the synthesis of red blood cells, white blood cells and blood platelets.

Myelofibrosis may lead to conditions such as increased or decreased the production of blood cells such as polycythemia vera, thrombocytopenia, and primary myelofibrosis. Together, these conditions are known as Philadelphia-chromosome-negative chronic myeloproliferative neoplasms.

The increased or decreased production of blood cells leads to either one or more comorbid conditions such as anemia, enlarged spleen, and liver impairment.

Myelofibrosis occurs among all age groups, but a considerable number of cases are observed in people above 50 years of age. A higher number of cases are also reported in children, with maximum cases reported in the female population.

Symptoms

The symptoms of myelofibrosis are not observed in the early onset of the disease. However, the symptoms tend to progressively develop over a period of time. The location of the tumor and the type of blood cell determines the severity of the condition.

The common symptoms of myelofibrosis include:

  • Fever
  • Bone pain
  • Decreased oxygen supply
  • Increased lethargy due to anemia
  • Decreased immunity
  • Increased night sweats
  • Increased bleeding and bruising
  • Pale skin
  • Weight loss

 

Causes

The exact cause of myelofibrosis is not known but it may occur due to gene mutation of the stem cells. The stem cells are responsible for the production and replication of red blood cells, white blood cells and platelets.

A mutation in the stem cells leads to a rapid multiplication of the blood cells that cause overcrowding of the blood cells in the bone marrow. This leads to altered levels of red blood cells, white blood cells and platelets.

A specific type of gene known as Janus kinase-2 (JAK-2) gene is responsible for myelofibrosis disorder.

Risk factors

The risk of developing myelofibrosis may increase with the following conditions:

 

  • Age: This condition is common among all age groups. However, people above the age of 50 years are at an increased risk of developing myelofibrosis
  • The secondary cause of other blood disorders: People suffering from thrombocytopenia and polycythemia vera are at an increased risk of developing myelofibrosis over a period of time
  • Exposure to certain chemicals: Frequent and long-term exposure to industrial chemicals such as benzene and toluene increase the risk of developing myelofibrosis
  • Exposure to radiation: Frequent exposure to high-intensity radiations from radioactive contrast material and atomic nuclear reactors are at risk of developing myelofibrosis

Complications

The complications that develop with myelofibrosis include:

  • Portal hypertension: It occurs due to the increased blood flow from the enlarged spleen into the liver. An increased blood flow in the portal veins leads to increased pressure on the blood vessels of the stomach and the intestines, leading to damage of blood vessels.
  • Bone pain and joint pains: It occurs due to the inflammation and hardening of the bones in the body causing immobility of the bones and joints. This leads to pain and inflammation of the joints and bones in the body.
  • Increased risk of bleeding: The progression of myelofibrosis leads to decreased blood platelets, a condition known as thrombocytopenia. A sharp decrease in the platelet count increases the risk of bleeding.

Diagnosis

Myelofibrosis is diagnosed by monitoring the following parameters:

 

  • Physical examination: The doctor physically examines the patient for parameters such as blood pressure, pulse rate, respiratory rate, swollen lymph nodes and abdominal tenderness to determine enlarged spleen and liver.
  • Complete blood cell profile: A complete blood cell count is taken to examine the abnormalities in red blood cells, white blood cells and platelets.
  • Bone marrow aspiration: The test is performed by the collection of stem cells of the bone marrow from the pelvic or the sternum of the chest bone. This helps in determining the morphology and the cell count of stem cells.
  • Image scan: Imaging scans such as magnetic resonance imaging (MRI) scan and x-ray scan helps in diagnosing any underlying cause of myelofibrosis and the degree of bone damage caused.
  • Genetic mutation test: The test evaluates the changes in the genes caused due to mutations.

Treatment

Myelofibrosis can be treated by the following treatment procedures:

 

  • Medication therapy: This therapy includes drugs which treat the symptoms caused due to JAK-2 gene mutations. It works by stopping the action of JAK-2 genes in healthy and diseased cells. Medication therapy also includes drugs to treat anemia and other blood cell disorders.
  • Bone marrow transplantation: The surgery is performed to replace the diseased cells with the healthy cells from a donor. The transplanted donor stem cells regenerate to produce new blood cells in the body. The doctor may recommend for chemotherapy or radiation therapy to destroy the diseased bone marrow cells. This helps in faster regeneration of newer stem cells in the body.
  • Chemotherapy: Cytotoxic agents are used to treat symptoms of enlarged spleen that cause pain and inflammation in the body.
  • Radiation therapy: High-intensity radiations are used to kill the cancer cells of the bone marrow. This therapy is recommended along with chemotherapy for better treatment.
  • Surgery: The surgery for spleen removal is performed to treat the enlarged spleen. The surgery is performed when other treatment procedures are not effective in treating myelofibrosis.

Management

The effective use of medications and other treatment options helps in the proper management of myelofibrosis.

Support from family and friends are essential to prevent the risk of getting depressed as the treatment requires a frequent hospital visit and longer duration of hospital stay.

Practicing yoga, meditation, and regular exercise helps in coping with the condition and also helps in better response rate.

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