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Myelofibrosis

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INTRODUCTION

Myelofibrosis (MF) is a type of blood cancer that is closely connected to myeloproliferative neoplasms (MPNs), in which the bone marrow cells that make the body’s blood cells develop and behave improperly. Excessive fibrous (scar) tissue production in the bone marrow is the outcome, which can cause severe anemia, weakness, weariness, enlargement of the spleen and liver, and bodily wasting (loss of body mass or size). 

MF can develop on its own or because of the advancement of other MPNs, such as polycythemia vera (PV) and essential thrombocythemia (ET). PV or ET are the starting points for 15 to 20 percent of MF cases. Agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, and myelosclerosis with myeloid metaplasia are some of the various names for MF. 

 

What is Myelofibrosis? 

Myelofibrosis is a rare bone marrow cancer that causes fibrous (scar) tissue to replace the marrow. The soft, fatty tissue inside the bones is known as bone marrow. When a mutation in the DNA of a single hematopoietic (blood-forming) stem cell occurs, cancer develops. Bone marrow stem cells can divide and multiply into a variety of specialized cells that make up the bone marrow and blood. The mutations are passed on to new cells as the mutated bone marrow cell replicates and divides. This abnormal cell production eventually outnumbers the bone marrow’s ability to generate enough normal blood cells, such as red blood cells, which transport oxygen to tissues; white blood cells, which fight infection; platelets, which aid in blood clotting. 

 

How Does Myelofibrosis Develop? 

MF develops in bone marrow stem cells after a genetic mutation. A mutation in the Janus kinase 2 gene (JAK2) affects 50 to 60 percent of patients with MF, while a mutation in the calreticulin gene affects about 25 percent. Several additional mutations have lately been discovered in MF patients. Other probable gene variants linked to MF are being investigated by researchers. The reason for the gene mutation or the disease is unknown.  MF generally progresses slowly, and some people may go years without experiencing any symptoms. Other patients’ bone marrow may deteriorate over time and require treatment. Patients must be monitored on a frequent basis in both circumstances. 

  

Types of Myelofibrosis 

The type of MPN is determined by the excess of red blood cells, white blood cells, or platelets. The body can create too many types of blood cells at times, although white blood cells are usually affected more than the others. There are six forms of chronic myeloproliferative neoplasms: 

  • Chronic myelogenous leukemia. 
  • Polycythemia vera. 
  • Primary myelofibrosis (also called chronic idiopathic myelofibrosis). 
  • Essential thrombocythemia. 
  • Chronic neutrophilic leukemia. 
  • Chronic eosinophilic leukemia.  

 

Sources:  

https://www.mayoclinic.org