It usually strikes after the age of 50. One of its most famous victims was Dave Fisher, lead singer of 1980s folk group known as the Highwaymen. According to the Washington Post, the group was best known for its recording of the Civil Way anti-slavery song Michael, Row the Boat Ashore. Fisher was 69 when he died of myelofibrosis.
Although it’s a disease of middle age or older, myelofibrosis can occur at any age. It’s a serious bone disorder that disrupts red blood cell production. Patients experience significant scarring in their bone marrow. This causes severe anemia, weakness and fatigue, the Mayo Clinic indicates.
The condition is also known as agnogenic myeloid metaplasia and idiopathic myelofibrosis.
Causes and risk factors
Myelofibrosis occurs as the result of a mutation of a single hematopoietic stem cell. This is a specialized cell in bone matter. Although experts don’t know what causes the mutation, they believe it occurs sometime after birth and isn’t a congenital condition.
As the affected cell divides, the mutation appears in new cells. Eventually, the individual has a shortage of red blood cells, causing anemia. He or she also has too many white blood cells with varying levels of platelets. Scientists believe that fibrosis – scarring – occurs within the bone marrow as a reaction to the activity of cells carrying the mutation.
Individuals who have been exposed to toxins like thorium dioxide, toluene, benzene and ionizing radiation are at an elevated risk for myelofibrosis. So are those who received the X-ray contrast substance Thorotrast in the 1930s and 1940s. Some patients show a mutation in their JAK2 MPL genes.
Except for one type of the disorder that tends to run in families, the illness rarely strikes children. Instead, it usually appears in individuals between 50 and 80.
Myelofibrosis develops slowly. Over time, most patients experience one or more standard symptoms. The most common is feeling tired, weak or short of breath due to the development of anemia.
Also common are pain and fullness below the left ribs due to an enlarged spleen, pale skin, an enlarged liver and easy bruising and bleeding. Some individuals complain of night sweats, fever, bone pain or frequent infections. Having any of these symptoms means it’s necessary to see a doctor.
Potential complications of myelofibrosis include ruptured veins requiring removal of the spleen, pain in the upper left side or shoulder, infections and bleeding issues. Formation of blood cells outside the bone marrow can create tumors made up of blood cells throughout the body.
The disorder can cause hardening of the bone narrow and connective tissue inflammation, resulting in severe bone and joint pain plus tenderness. The condition is also associated with the development of gout and acute myelogenous leukemia.
Treatment and prognosis
Most myelofibrosis patients get progressively worse. Some individuals develop leukemia. Others are able to live without symptoms for years.
Fortunately, as long as patients don’t show signs of anemia, an enlarged spleen or other complications, doctors usually don’t prescribe any treatment. For those who need them, there are many treatment options. The most common are blood transfusions, androgen therapy, chemotherapy, radiation, thalidomide with steroids and removal of the spleen.
A stem cell transplant is the only treatment that can potentially cure this condition. Researchers are working on a reduced-intensity transplant that uses lower doses of chemotherapy and radiation before the procedure. This technique instead relies on the donor’s immune system to destroy any diseased cells. Doctors hope it will prove equally effective but safer than standard transplant procedures for treating myelofibrosis.
Washington Post site
Mayo Clinic site