Top 15 Symptoms of Myelofibrosis

The Main Symptoms of Myelofibrosis  that many are unaware of. Also, myelofibrosis  is a rare type of cancer in which the bone marrow (the soft, spongy tissue inside most bones) is replaced by fibrous scar tissue. It is considered a form of chronic leukemia. When myelofibrosis  occurs on its own, it is called primary myelofibrosis  . If it occurs as a result of another bone marrow disease, it is known as secondary myelofibrosis  . Bone marrow contains immature blood-forming cells that can turn into three types of specialized blood cells: red blood cells, white blood cells, or platelets.

When a change (mutation) occurs in the DNA of a single cell, the mutation will be passed on to new cells when the defective cell divides. As time passes, more and more abnormal cells are produced. They can also gain additional mutations making cancer cells more aggressive over time.

They eventually replace normal cells in the bone marrow. The bone marrow begins to lose the ability to produce normal blood cells. Abnormal blood-forming cells can also grow outside the bone marrow in the liver, lungs, lymph nodes, and other organs.

Myelofibrosis is a rare  condition, with about 1.5 cases reported per 100,000 people per year in Brazil. This occurs in both men and women. People of any age can have myelofibrosis , although it is more likely to be diagnosed in people over 50. For children diagnosed with myelofibrosis , onset is usually before age 3 years.

Causes of Myelofibrosis:  The underlying cause ofprimary myelofibrosis  has not yet been determined. Although myelofibrosis  is not inherited, it is associated with DNA changes in certain genes. Proteins called Janus-associated kinases (JAKs) play a role in myelofibrosis . JAKs regulate the production of blood cells in the bone marrow, signaling the cells to divide and grow. If the JAKs become hyperactive, many or small blood cells will be produced.

About 50% of patients have a mutation in the JAK2 gene. Another 5-10% of patients have a mutation in the myeloproliferative leukemia (MPL) gene. A mutation called calreticulin (CALR) accounts for about 35% of cases of meofibrosis. Risk factors for myelofibrosis  include exposure to ionizing radiation or petrochemicals such as benzene or toluene.

When myelofibrosis  occurs on its own, it is called primary myelofibrosis  . Secondary myelofibrosis  can develop in patients with other bone marrow cancers, such as essential thrombocythemia (excess platelet production) or polycythemia vera (excess red blood cell production). This is known as post-essential thrombocythemia or post-polycythemia vera for Myelofibrosis . So, check now  The Top 15 Symptoms of Myelofibrosis:

Symptoms of Myelofibrosis:  A person with Myelofibrosis  may not have symptoms for many years. About a third of patients have no symptoms during the early stages of the disease. However, during the course of the disease, they may experience the following symptoms or conditions. The main symptoms of  myelofibrosis include:

• Anemia  – A deficiency of red blood cells, often accompanied by fatigue, weakness, and shortness of breath
• Pale skin
• Enlarged spleen (splenomegaly) – This can result in a feeling of fullness or discomfort in the upper left part of the abdomen
• Portal hypertension – Increased blood pressure in the vein that carries blood from the spleen to the liver
• Enlarged veins in the stomach and esophagus – These veins can rupture and cause bleeding
• night sweat
• unexplained blood clots
• frequent infections
• Fever
• Itch
• Abnormal bleeding or bruising
• enlarged liver
• Wing or joint pain
• Weight loss
• Extramedullary hematopoiesis – Abnormal growth of blood-forming cells outside the bone marrow.

Diagnosis of Myelofibrosis:  Your doctor will perform a physical exam and ask about your medical history, including any symptoms you are experiencing. The doctor will check for signs of an enlarged spleen or anemia  . Diagnostic tests for Myelofibrosis  may be performed, including:

• Complete blood count (CBC) – An elevated number of white blood cells and platelets and a lower number of normal red blood cells can suggest myelofibrosis .
• Blood tests: Elevated levels of uric acid, bilirubin, and lactic dehydrogenase can mark the presence of myelofibrosis .

Additional tests may be needed to confirm the diagnosis of  myelofibrosis , including:

• Gene mutation analysis – Blood cells can be screened for certain mutations associated with myelofibrosis .
• Bone marrow biopsy – The bone marrow sample can be removed for examination under a microscope.
• Imaging tests: An ultrasound test may be performed to check for an enlarged spleen.

Myelofibrosis Treatments:  Various methods are available to treat myelofibrosis , depending on the patient’s symptoms and individual circumstances. Some people may not have symptoms for years. Even if they do not require immediate treatment, they should be monitored for changes that worsen the condition. For most patients, the goal is to manage conditions associated with myelofibrosis .

• Jakafi (ruxolitinib) is the first drug for the treatment of intermediate or high-risk myelofibrosis  . It is a multikinase inhibitor that works to suppress Janus-associated kinase-associated (JAK) signaling. The medication is taken orally. This helps alleviate some of the symptoms associated with the condition, including an enlarged spleen, night sweats, itching, weight loss, and fever .

Medications used to treat anemia  include:

• glucocorticoids
• Androgens (synthetic male hormones)
• Immunomodulators – Interferon is given through intramuscular or subcutaneous (under the skin) injections. Thalidomide and lenalidomide are taken by mouth. Pomalidomide is being investigated in clinical trials for the treatment of anemia  associated with myelofibrosis .
• Chemotherapy Medications – Hydroidurexia is given orally and cladribine is given intravenously.
• Blood transfusions may be given to increase the number of red blood cells in patients with  severe anemia .

Medications used to treat an enlarged spleen (splenomegaly) include

• Jakafi
• hydroxyurea
• Cladribine
• interferon

In severe cases, removal of the spleen (splenectomy) or radiation therapy may be necessary. Radiation therapy can also be used to treat extramedullary hematopoiesis (abnormal growth of blood-forming cells outside the bone marrow). Thrombosis (blood clots) can be prevented by taking low doses of aspirin or hydroxyurea.

Prevention of Myelofibrosis:  Myelofibrosis  cannot be prevented, but allogeneic hematopoietic cell transplantation (HCT) may be a potential cure. The procedure involves replacing a patient’s immune system with a suitable donor. This new immune system seeks out and kills cancer cells in the myelofibrosis patient’s bone marrow , in addition to providing healthy bone marrow cells.

Immediately before the transplant, the patient must undergo chemotherapy and/or radiation therapy to suppress their own immune system so that the donor’s cells will take over. HCT carries a high risk of complications and is only suitable for certain patients. The risk of a complication is higher for people with other medical conditions. Several factors must be taken into account for patients who may be candidates for the procedure, including their age.