The blood disorder, mylofibrosis begins in the bone marrow and is a disorder that can disrupt the normal production of blood cells within your body. It is a rare disorder that typically occurs between the ages of 50 and 70.
Those with the disorder develop anemia that progressively becomes worse over time. Red blood cells are immature or they are produced in a misshapen manner. White blood cells and platelets are also formed in an immature fashion.
As the disorder progresses what usually happens is that the platelet count typically will decrease while the white blood cell count may increase or decrease. When more cells are manufactured the liver and spleen will enlarge to accommodate them as they make more cells in response to the lack of cells being manufactured in the bone marrow.
Unfortunately death usually occurs 3 to 5 years from diagnosis. Stem cell transplantation is hope for those with this disorder.
The cause of myelofibrosis is not currently known. Individuals may have other blood disorders when they are diagnosed with myelofibrosis such as myelocytic leukemia, lymphoma, or multiple myeloma, polycythemia vera, or thrombocythemia.
Sometimes myelofibrosis is seen in those that have tuberculosis or have bone infections. Individuals who have been exposed to certain toxic substances, such as benzene or radiation, have an increased risk for developing myelofibrosis.
The symptoms of myelofibrosis are anemia, low-grade fever, and night sweats. Individuals may also experience frequent infections due to low white cell counts. Due to low platelet counts they are also more likely to experience incidents of excessive bleeding. They may feel abdominal discomfort as the liver or spleen enlarges.
They may also experience portal hypertension, which is when the blood pressure in veins of the liver becomes abnormally high. They may also have esophageal varices, which is when varicose veins in the esophagus bleed. Under microscopic examination a person’s red blood cells are misshapen and immature.
A diagnosis of myelofibrosis is made by examining the red blood cells on microscopy and by performing a bone marrow biopsy.
Treatment for Myelofibrosis
The only treatment that may affect a cure is stem cell transplantation. Individuals with the disease older than age 75 rarely are recommended for stem cell transplantation due to the nature of the procedure.
There are treatments that can delay or bring relief from symptoms such as hormone therapy, blood transfusions to treat the anemia and chemotherapy to reduce the size of the liver or spleen.
Myelofibrosis is a progressive blood disorder in which depending on the age and health of the individual at the time of diagnosis live anywhere from 1 year to 5 years after diagnosis without a stem cell transplant. This blood disorder is devastating to the individual with the disorder and to their family members and other loved ones.