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Myelofibrosis

Myelofibrosis

Definition

Myelofibrosis is a rare disease of the bone marrow in which collagen builds up fibrous scar tissue inside the marrow cavity. This is caused by the uncontrolled growth of a blood cell precursor, which results in the accumulation of scar tissue in bone marrow. Myelofibrosis goes by many names including idiopathic myelofibrosis, agnogenic myeloid metaplasia, chronic myelosclerosis, aleukemic megakaryocytic myelosis, and leukoerythroblastosis.

Description

Myelofibrosis can be associated with many other conditions including breast cancer , prostate cancer , Hodgkin's disease , non-Hodgkin's lymphomas , acute myelocytic leukemia , acute lymphocytic leukemia , hairy cell leukemia , multiple myeloma , myeloproliferative diseases , tuberculosis, Gaucher's disease, and Paget's disease of bone. Myelofibrosis typically becomes progressively worse and can cause death.

In myelofibrosis, abnormal cells (hematopoietic stem cells) grow out of control and begin to produce both immature blood cells and excess scar (fibrous) tissue. The fibrous tissue builds up (fibrosis) primarily in the bone marrow, the place where blood cells are produced. The fibrous tissue interferes with the production of normal blood cells. The outcome of this is that the blood made by the bone marrow is of poor quality. To compensate for this, blood cell production occurs in other parts of the body (extramedullary hematopoiesis), but most notably in the spleen and liver. This causes enlargement of the spleen (splenomegaly) and the liver (hepatomegaly). Extramedullary hematopoiesis is not effective and, combined with the reduced production of blood cells by the bone marrow, a condition called anemia results.

The abnormal stem cells can spread throughout the body, settle in other organs, and form tumors that produce more abnormal blood cells and fibrous tissue. These tumors are most commonly found in the adrenals, kidneys, lymph nodes, breast, lungs, skin, bowel, thymus, thyroid, prostate, and urinary tract.

Demographics

Most patients with myelofibrosis are over 50 years old; the average age at diagnosis is 65 years. However, myelofibrosis can occur at any age. Myelofibrosis occurs with equal frequency in women and men, but in children it affects girls twice as often as it does boys.

Causes and symptoms

Myelofibrosis is caused by an abnormality in a single stem cell, which causes it to grow out of control. Myelofibrosis tumors that have originated from a single cell are called monoclonal. The cause of the stem cell abnormality is unknown. Persons who were exposed to benzene or high doses of radiation have developed myelofibrosis. There may be an association between myelofibrosis and autoimmune diseases, such as systemic lupus erythematosus and scleroderma, in which the immune system treats certain molecules of the body as foreign invaders.

Symptoms usually appear slowly over a long period of time. About one quarter of all patients with myelofibrosis have no symptoms (asymptomatic). An enlarged spleen discovered at an annual medical examination may be the first clue. Symptoms of myelofibrosis include:

  • fatigue
  • weight loss
  • paleness
  • fever
  • sweating
  • weakness
  • heart palpitations
  • shortness of breath
  • itching
  • feeling full after eating a small amount of food
  • stomach pain or discomfort
  • pain in the left shoulder or upper left portion of the body
  • unexpected bleeding
  • bone pain , especially in the legs

Diagnosis

Because symptoms are similar to other diseases (mostly leukemias), myelofibrosis is not easy to diagnose. The doctor would use his or her hands to feel (palpate) for enlargement of the spleen and liver. Blood tests and urine tests would be performed. Bone marrow aspiration and biopsy can help make a diagnosis, but they often fail because of the fibrosis. X-ray imaging and magnetic resonance imaging (MRI) may be performed.

Treatment

Many asymptomatic patients, if stable, do not require treatment. There is no cure for myelofibrosis, although bone marrow transplantation is curative in some cases. Treatment is aimed at reducing symptoms and improving quality of life.

Medications

Male hormones (androgens) can be used to treat anemia but, in women, these drugs can cause the development of male characteristics (e.g., hair growth on the face and body). Glucocorticoid therapy is also an effective treatment of anemia and can improve myelofibrosis in children. Nutrients that stimulate blood formation (hematinics), such as iron, folic acid , and vitamin B12, may reduce anemia. Cancer chemotherapy (usually hydroxyurea ) can decrease splenomegaly and hepatomegaly, reduce symptoms of myelofibrosis, lessen anemia, and sometimes reduce bone marrow fibrosis. The bone marrow of myelofibrosis patients is often not strong enough to withstand the harsh chemotherapy drugs, so this treatment is not always an option. Interferon-alpha has been shown to reduce spleen size, reduce bone pain, and, in some cases, increase the number of blood platelets (structures involved in blood clotting).

Other treatments

In certain cases, the enlarged spleen may be removed (splenectomy ). Conditions that warrant splenectomy include spleen pain, the need for frequent blood transfusion, very low levels of platelets (thrombocytopenia ), and extreme pressure in the blood vessels of the liver (portal hypertension).

Radiation therapy is used to treat splenomegaly, spleen pain, bone pain, tumors in certain places such as next to the spinal cord, and fluid accumulation inside the abdomen (ascites ). Patients who are not strong enough to undergo splenectomy are often treated with radiation therapy.

Bone marrow transplantation may be used to treat some patients with myelofibrosis. This procedure may be performed on patients who are less than 50 years old, have a poor life expectancy, and have a brother or sister with blood-type similarities.

Patients with severe anemia may require blood transfusions.

Prognosis

Similar to leukemias, myelofibrosis is progressive and often requires therapy to control the disease. Myelofibrosis can progress to acute lymphocytic leukemia or lymphoma. Although a number of factors to predict the survival time have been proposed, advanced age or severe anemia are consistently associated with a poor prognosis. The average survival rate of patients diagnosed with myelofibrosis is five years. Death is usually caused by infection, bleeding, complications of splenectomy, heart failure, or progression to leukemia. Spontaneous remission is rare.

Prevention

Persons who have been exposed to radiation, benzene, or radioactive thorium dioxide (a chemical used during certain diagnostic radiological procedures) are at risk for myelofibrosis.

Resources

BOOKS

Lichtman, Marshall. "Idiopathic Myelofibrosis (Agnogenic Myeloid Metaplasia)." In Williams Hematology, edited by Ernest Beutler et al. New York: McGraw Hill, 2001, pp.1125-36.

Mavroudis, Dimitrios and John Barrett. "Myelofibrosis (Agno genic Myeloid Metaplasia)." In Bone Marrow Failure Syndromes, edited by Neal Young. Philadelphia: W.B. Saunders Company, 2000, pp.122-34.

Peterson, Powers. "Myelofibrosis." In Practical Diagnosis of Hematologic Disorders, edited by Carl Kjeldsberg. Chicago: ASCP Press, 2000, pp.477-9.

PERIODICALS

Reilly, J.T. "Idiopathic Myelofibrosis: Pathogenesis, Natural History and Management." Blood Reviews 11 (1997): 233-42.

Belinda Rowland, Ph.D.

J. Ricker Polsdorfer, M.D.

KEY TERMS

Anemia

Low numbers of red blood cells in the blood.

Benzene

A colorless volatile flammable toxic liquid hydrocarbon used as a solvent and as a motor fuel.

Biopsy

Surgical removal of tissue for microscopic examination.

Fibrosis

Buildup of scar tissue.

Glucocorticoid therapy

Treatment using corticoids that are anti-inflammatory and immunosuppressive.

Leukemia

Cancer of white blood cells.

Portal hypertension

Extreme pressure on the blood vessels of the liver.

Stem cell

A cell that has the ability to become many different specialized cells.

Cite this article
Pick a style below, and copy the text for your bibliography.

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Rowland, Belinda; Polsdorfer, J. Ricker. "Myelofibrosis." Gale Encyclopedia of Cancer. 2002. Encyclopedia.com. 30 Sep. 2016 <http://www.encyclopedia.com>.

Rowland, Belinda; Polsdorfer, J. Ricker. "Myelofibrosis." Gale Encyclopedia of Cancer. 2002. Encyclopedia.com. (September 30, 2016). http://www.encyclopedia.com/doc/1G2-3405200321.html

Rowland, Belinda; Polsdorfer, J. Ricker. "Myelofibrosis." Gale Encyclopedia of Cancer. 2002. Retrieved September 30, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3405200321.html

Myelofibrosis

Myelofibrosis

Definition

Myelofibrosis is a rare disease of the bone marrow in which collagen builds up fibrous scar tissue inside the marrow cavity. This is caused by the uncontrolled growth of a blood cell precursor, which results in the accumulation of scar tissue in bone marrow. Myelofibrosis goes by many names including idiopathic myelofibrosis, agnogenic myeloid metaplasia, chronic myelosclerosis, aleukemic megakaryocytic myelosis, and leukoerythroblastosis.

Description

Myelofibrosis can be associated with many other conditions including breast cancer, prostate cancer, Hodgkin's disease, non-Hodgkin's lymphoma, acute myeloid leukemia, acute lymphocytic leukemia, hairy cell leukemia, multiple myeloma, myeloproliferative diseases, tuberculosis, Gaucher's disease, and Paget's disease of bone. Myelofibrosis typically becomes progressively worse and can cause death.

In myelofibrosis, abnormal cells (hematopoietic stem cells) grow out of control and begin to produce both immature blood cells and excess scar (fibrous) tissue. The fibrous tissue builds up (fibrosis) primarily in the bone marrow, the place where blood cells are produced. The fibrous tissue interferes with the production of normal blood cells. The outcome of this is that the blood made by the bone marrow is of poor quality. To compensate for this, blood cell production occurs in other parts of the body (extramedullary hematopoiesis), but most notably in the spleen and liver. This causes enlargement of the spleen (splenomegaly) and the liver (hepatomegaly). Extramedullary hematopoiesis is not effective and, combined with the reduced production of blood cells by the bone marrow, a condition called anemia results.

The abnormal stem cells can spread throughout the body, settle in other organs, and form tumors that produce more abnormal blood cells and fibrous tissue. These tumors are most commonly found in the adrenals, kidneys, lymph nodes, breast, lungs, skin, bowel, thymus, thyroid, prostate, and urinary tract.

Most patients with myelofibrosis are over 50 years old; the average age at diagnosis is 65 years. However, myelofibrosis can occur at any age. Myelofibrosis occurs with equal frequency in women and men, but in children it affects girls twice as often as it does boys.

Causes and symptoms

Myelofibrosis is caused by an abnormality in a single stem cell, which causes it to grow out of control. Myelofibrosis tumors that have originated from a single cell are called monoclonal. The cause of the stem cell abnormality is unknown. Persons who were exposed to benzene or high doses of radiation have developed myelofibrosis. There may be an association between myelofibrosis and autoimmune diseases, such as systemic lupus erythematosus and scleroderma, in which the immune system treats certain molecules of the body as foreign invaders.

Symptoms usually appear slowly over a long period of time. About one quarter of all patients with myelofibrosis have no symptoms (asymptomatic). An enlarged spleen discovered at an annual medical examination may be the first clue. Symptoms of myelofibrosis include:

  • fatigue
  • weight loss
  • paleness
  • fever
  • sweating
  • weakness
  • heart palpitations
  • shortness of breath
  • itchiness
  • feeling full after eating a small amount of food
  • stomach pain or discomfort
  • pain in the left shoulder or upper left portion of the body
  • unexpected bleeding
  • bone pain, especially in the legs

Diagnosis

Because symptoms are similar to other diseases (mostly leukemias), myelofibrosis is not easy to diagnose. The doctor would use his or her hands to feel (palpate) for enlargement of the spleen and liver. Blood tests and urine tests would be performed. Bone marrow aspiration and biopsy can help make a diagnosis, but they often fail because of the fibrosis. X-ray imaging and magnetic resonance imaging (MRI) may be performed.

Treatment

Many asymptomatic patients, if stable, do not require treatment. There is no cure for myelofibrosis, although bone marrow transplantation is curative in some cases. Treatment is aimed at reducing symptoms and improving quality of life.

Medications

Male hormones (androgens) can be used to treat anemia but, in women, these drugs can cause the development of male characteristics (e.g., hair growth on the face and body). Glucocorticoid therapy is also an effective treatment of anemia and can improve myelofibrosis in children. Nutrients that stimulate blood formation (hematinics), such as iron, folic acid, and vitamin B12, may reduce anemia. Cancer chemotherapy (usually hydroxyurea) can decrease splenomegaly and hepatomegaly, reduce symptoms of myelofibrosis, lessen anemia, and sometimes reduce bone marrow fibrosis. The bone marrow of myelofibrosis patients is often not strong enough to withstand the harsh chemotherapy drugs, so this treatment is not always an option. Interferonalpha has been shown to reduce spleen size, reduce bone pain, and, in some cases, increase the number of blood platelets (structures involved in blood clotting).

Other treatments

In certain cases, the enlarged spleen may be removed (splenectomy ). Conditions that warrant splenectomy include spleen pain, the need for frequent blood transfusion, very low levels of platelets (thrombocytopenia ), and extreme pressure in the blood vessels of the liver (portal hypertension ).

Radiation therapy is used to treat splenomegaly, spleen pain, bone pain, tumors in certain places such as next to the spinal cord, and fluid accumulation inside the abdomen (ascites ). Patients who are not strong enough to undergo splenectomy are often treated with radiation therapy.

Bone marrow transplantation may be used to treat some patients with myelofibrosis. This procedure may be performed on patients who are less than 50 years old, have a poor life expectancy, and have a brother or sister with blood-type similarities.

Patients with severe anemia may require blood transfusions.

Prognosis

Similar to leukemias, myelofibrosis is progressive and often requires therapy to control the disease. Myelofibrosis can progress to acute lymphocytic leukemia or lymphoma. Although a number of factors to predict the survival time have been proposed, advanced age or severe anemia are consistently associated with a poor prognosis. The average survival rate of patients diagnosed with myelofibrosis is five years. Death is usually caused by infection, bleeding, complications of splenectomy, heart failure, or progression to leukemia. Spontaneous remission is rare.

KEY TERMS

Anemia Low numbers of red blood cells in the blood.

Benzene A colorless volatile flammable toxic liquid hydrocarbon used as a solvent and as a motor fuel.

Biopsy Surgical removal of tissue for microscopic examination.

Fibrosis Buildup of scar tissue.

Glucocorticoid therapy Treatment using corticoids that are anti-inflammatory and immunosuppressive.

Leukemia Cancer of white blood cells.

Portal hypertension Extreme pressure on the blood vessels of the liver.

Stem cell A cell that has the ability to become many different specialized cells.

Prevention

Persons who have been exposed to radiation, benzene, or radioactive thorium dioxide (a chemical used during certain diagnostic radiological procedures) are at risk for myelofibrosis.

Resources

BOOKS

Lichtman, Marshall. "Idiopathic Myelofibrosis (Agnogenic Myeloid Metaplasia)." In Williams Hematology, edited by Ernest Beutler, et al. New York: McGraw Hill, 2001, pp.1125-36.

Mavroudis, Dimitrios and John Barrett. "Myelofibrosis (Agnogenic Myeloid Metaplasia)." In Bone Marrow Failure Syndromes, edited by Neal Young. Philadelphia: W.B. Saunders Company, 2000, pp.122-34.

Peterson, Powers. "Myelofibrosis." In Practical Diagnosis of Hematologic Disorders, edited by Carl Kjeldsberg. Chicago: ASCP Press, 2000, pp.477-9.

Cite this article
Pick a style below, and copy the text for your bibliography.

  • MLA
  • Chicago
  • APA

Rowland, Belinda; Polsdorfer, J.. "Myelofibrosis." Gale Encyclopedia of Medicine, 3rd ed.. 2006. Encyclopedia.com. 30 Sep. 2016 <http://www.encyclopedia.com>.

Rowland, Belinda; Polsdorfer, J.. "Myelofibrosis." Gale Encyclopedia of Medicine, 3rd ed.. 2006. Encyclopedia.com. (September 30, 2016). http://www.encyclopedia.com/doc/1G2-3451601095.html

Rowland, Belinda; Polsdorfer, J.. "Myelofibrosis." Gale Encyclopedia of Medicine, 3rd ed.. 2006. Retrieved September 30, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3451601095.html

myelofibrosis

myelofibrosis (my-ĕ-loh-fy-broh-sis) n. a chronic but progressive disease characterized by fibrosis of the bone marrow, which leads to anaemia; enlargement of the spleen; and the presence of myeloid tissue in abnormal sites, such as the spleen and liver. Its cause is unknown.

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"myelofibrosis." A Dictionary of Nursing. 2008. Encyclopedia.com. 30 Sep. 2016 <http://www.encyclopedia.com>.

"myelofibrosis." A Dictionary of Nursing. 2008. Encyclopedia.com. (September 30, 2016). http://www.encyclopedia.com/doc/1O62-myelofibrosis.html

"myelofibrosis." A Dictionary of Nursing. 2008. Retrieved September 30, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1O62-myelofibrosis.html

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