About

Myelofibrosis (MF)


About Myelofibrosis

Myelofibrosis is a rare condition that can occur in both men and women, primarily in people at least 60 years old. While children and young adults may also develop myelofibrosis, it is extremely uncommon. Present statistics report about 1.5 cases diagnosed annually per every 100,000 people in the United States.


MF originates in the bone marrow, where it causes fibrous scar tissue to replace the healthy, soft and spongy tissue that is normally inside your bones. These scars, called fibrosis, affect how many normal blood cells (such as red blood cells and white blood cells) your body can produce. Myelofibrosis is generally a slow growing cancer, and many people are able to live with it for years without detection. Yet sometimes MF grows more rapidly and requires prompt treatment.

A main characteristic of myelofibrosis is the overproduction of megakaryocytes, giant cells that normally release platelets in your body. The production of megakaryocytes is accompanied by the release of cytokines, proteins that typically make you feel sick. All of these cytokines lead to inflammation and more fibrosis in the bone marrow.

The underlying cause of myelofibrosis remains unknown, although some risk factors have been associated with this disease, including:

  • Age: MF is most commonly diagnosed in people over 60
  • Environment: exposure to ionizing radiation and petrochemicals, such as benzene and toluene, may increase the risk of developing MF
  • JAK2 mutation: about 50% to 60% of people with myelofibrosis have a mutation in the JAK2 gene; this mutation instructs blood cells to multiply and divide even when the body doesn’t need more red blood cells
  • CALR mutation: approximately 35% of people with MF have a CALR (calreticulin) genetic mutation

MF grows very slowly, which is why many people with this blood cancer don’t feel symptoms for years. Over time, as MF affects the way your body makes red blood cells, white blood cells, and platelets, the following symptoms usually present:

  • Anemia: a shortage of red blood cells; generally leads to fatigue, weakness, and shortness of breath
  • Pale skin
  • Frequent infections
  • Fever
  • Itching
  • Abnormal bleeding or bruising
  • Enlarged liver
  • Bone or joint pain
  • Night sweats
  • Weight loss
  • Enlarged spleen (splenomegaly): causes discomfort or a feeling of fullness in the upper left part of the abdomen
  • Portal hypertension: an increase in blood pressure in the vein that goes from the spleen to the liver
  • Dilated veins in the stomach and esophagus: these veins may rupture and lead to bleeding
  • Unexplained blood clots
  • Extramedullary hematopoiesis: abnormal growth of blood-forming cells outside the bone marrow. These cells can form tumors that compress body organs or interfere with their function

MF is regarded as a rare form of chronic leukemia. When this disease occurs on its own, it is classified as primary myelofibrosis. If it occurs as the result of another disease of the bone marrow (such as essential thrombocythemia or polycythemia) it is classified as secondary myelofibrosis.

In about 12% of all primary myelofibrosis cases, the disease will progress to acute myeloid leukemia, which is generally a very aggressive form of cancer.


What is myelofibrosis?

MF originates in the bone marrow, where it causes fibrous scar tissue to replace the healthy, soft and spongy tissue that is normally inside your bones. These scars, called fibrosis, affect how many normal blood cells (such as red blood cells and white blood cells) your body can produce. Myelofibrosis is generally a slow growing cancer, and many people are able to live with it for years without detection. Yet sometimes MF grows more rapidly and requires prompt treatment.

A main characteristic of myelofibrosis is the overproduction of megakaryocytes, giant cells that normally release platelets in your body. The production of megakaryocytes is accompanied by the release of cytokines, proteins that typically make you feel sick. All of these cytokines lead to inflammation and more fibrosis in the bone marrow.

 

What are the risk factors for myelofibrosis?

The underlying cause of myelofibrosis remains unknown, although some risk factors have been associated with this disease, including:

  • Age: MF is most commonly diagnosed in people over 60
  • Environment: exposure to ionizing radiation and petrochemicals, such as benzene and toluene, may increase the risk of developing MF
  • JAK2 mutation: about 50% to 60% of people with myelofibrosis have a mutation in the JAK2 gene; this mutation instructs blood cells to multiply and divide even when the body doesn’t need more red blood cells
  • CALR mutation: approximately 35% of people with MF have a CALR (calreticulin) genetic mutation

 

What are the symptoms of myelofibrosis?

MF grows very slowly, which is why many people with this blood cancer don’t feel symptoms for years. Over time, as MF affects the way your body makes red blood cells, white blood cells, and platelets, the following symptoms usually present:

  • Anemia: a shortage of red blood cells; generally leads to fatigue, weakness, and shortness of breath
  • Pale skin
  • Frequent infections
  • Fever
  • Itching
  • Abnormal bleeding or bruising
  • Enlarged liver
  • Bone or joint pain
  • Night sweats
  • Weight loss
  • Enlarged spleen (splenomegaly): causes discomfort or a feeling of fullness in the upper left part of the abdomen
  • Portal hypertension: an increase in blood pressure in the vein that goes from the spleen to the liver
  • Dilated veins in the stomach and esophagus: these veins may rupture and lead to bleeding
  • Unexplained blood clots
  • Extramedullary hematopoiesis: abnormal growth of blood-forming cells outside the bone marrow. These cells can form tumors that compress body organs or interfere with their function

 

Are there different types of myelofibrosis?

MF is regarded as a rare form of chronic leukemia. When this disease occurs on its own, it is classified as primary myelofibrosis. If it occurs as the result of another disease of the bone marrow (such as essential thrombocythemia or polycythemia) it is classified as secondary myelofibrosis.

In about 12% of all primary myelofibrosis cases, the disease will progress to acute myeloid leukemia, which is generally a very aggressive form of cancer.
 

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