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Cytopenic Myelofibrosis
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What is Cytopenic Myelofibrosis?

Introduction

Cytopenic Myelofibrosis, also known as CMF, is a rare type of blood cancer that affects the bone marrow and blood. It causes an abnormal increase in plasma cells (a type of white blood cell) in the bone marrow and spleen. The disease affects men more often than women and usually occurs between ages 40 to 60 years old.

Overview

Cytopenic myelofibrosis (CMF) is a rare blood disease that affects people with myeloproliferative neoplasms (MPNs). CMF occurs when the bone marrow produces too many immature white blood cells, which can lead to serious bleeding and infections.

To understand what cytopenic myelofibrosis is, it helps to know what myelofibrosis is and how it’s diagnosed. Myelofibrosis happens when there are too few red blood cells in the body and too many platelets—thrombocytes—that help with clotting. The cause of this condition is not known, but it may be related to an inherited genetic defect or exposure to radiation or certain medications.

Myeloproliferative neoplasms include chronic idiopathic myelofibrosis (CIM), polycythemia vera (PCV) and essential thrombocythemia (ET). These illnesses affect different parts of the bone marrow differently; however all three conditions can cause both overgrowth of white blood cells called leukocytosis as well as underproduction of red blood cells called anemia.

Symptoms

Symptoms of CMF include fatigue, weakness, weight loss and bone pain. Other symptoms include muscle pain and fever. It is important to note that the symptoms of CMF can be similar to those of other medical conditions. If you have any concerns about your health or if you experience any new symptoms that do not go away after a few days or weeks, please contact your doctor immediately.

Causes

Causes

A variety of factors can cause CMF. These include:

  • Hereditary (hereditary): If you have an inherited gene mutation, you’re at increased risk of developing CMF. It’s thought that many hereditary cases are due to mutations in the JAK2V617F gene, but other genetic mutations may also play a role in this disease.
  • Acquired: If you develop CMF after receiving certain drugs or radiation therapy to treat another condition, it’s called acquired myelofibrosis. Drugs that have been known to cause AML/CMML include hydroxyurea and methotrexate for cancer treatments; nitrofurantoin (Macrodantin), phenytoin (Dilantin), and phenobarbital for seizures; amphotericin B for fungal infections; paclitaxel or docetaxel chemotherapy drugs; tamoxifen or raloxifene hormone therapy pills used after menopause; and zidovudine (AZT) taken to prevent HIV infection. Radiation treatments that may contribute include radiation therapy given before a bone marrow transplantation procedure or during radiation therapy treatment for cancer .

Risk Factors

  • Heredity: Cytopenic myelofibrosis is a rare disease, but it does have a hereditary component.
  • Age: Most people with CMF develop symptoms after age 50.
  • Sex: Men are more likely than women to develop CMF, although this may be due to their higher rate of heavy alcohol consumption and smoking.
  • Race/Ethnicity: Caucasians of European descent have the highest rate of CMF compared to other races/ethnicities like Asians and African Americans.
  • Family history: People who have a family member who had CMF are more likely than those without family history to develop it themselves. This can affect future generations even if they themselves don’t have CMF (for example, if you were born before your father was diagnosed with CMF but later developed your own signs).

Complications

There are several complications that may occur in cytopenic myelofibrosis, including:

  • Cytopenia. This is a condition in which the number of red blood cells, white blood cells, and platelets decreases below normal levels.
  • Bone marrow fibrosis. When the bone marrow doesn’t produce enough healthy red blood cells to maintain a normal level of hemoglobin within the body because it’s been damaged by fibrosis (scarring), anemia develops as a result of this decreased production. This can be treated with transfusions that include healthy red blood cells or medications like EPO (erythropoietin) injections to stimulate red cell production within the body’s bone marrow.* Hyperviscosity syndrome. In this complication, high levels of proteins cause plasma to thicken within your circulatory system and reduce its ability to circulate oxygen throughout your body.* Sudden death due to heart failure or other causes

Tests And Diagnosis

The diagnosis of CMF is made by a combination of blood tests, imaging tests and a bone marrow biopsy.

The first step in diagnosing CMF is to get a CBC (complete blood count) which measures the red blood cells, white blood cells and platelets in your body. This is done because most people with CMF have low red blood cell counts.

A bone marrow biopsy may also be performed to confirm the diagnosis of myelofibrosis and determine if it has progressed to CMF (myelofibrosis).

If the doctor suspects that you have hyperviscosity syndrome, he or she will order an MRI scan or CT scan to look for enlarged liver or spleen.

Treatment And Drugs

Treatment depends on the severity of the disease. Treatment can include chemotherapy, stem cell transplant, or a combination of the two.

Learn about Cytopenic Myelofibrosis today.

Cytopenic Myelofibrosis is a rare disease that can be treated with drugs. It can be treated with a bone marrow transplant, stem cell transplant, blood transfusion and chemotherapy.

Conclusion

If you have symptoms of myelofibrosis, talk to your doctor. You can also learn more about cytopenic myelofibrosis by reading this article.

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