What is a Myeloproliferative Neoplasm?

Understanding MPNs: Rare Blood Cancers of the Bone Marrow

Myeloproliferative neoplasms (MPNs) are rare medical conditions, potentially life-threatening blood cancers that occur when your bone marrow starts producing excess blood cells, and it contains red blood cells, platelets and white blood cells. These extra cells can assemble in the blood or in bone marrow, leading to various critical health conditions. It is developed in the spongy tissues inside the bones. 'Myelo' refers to the bone marrow, and 'proliferative' means rapid growth of blood cells, and 'neoplasm' describes uncontrolled or abnormal growth. There is something that goes wrong in the production process of blood cells when people have myeloproliferative neoplasms. The overproduction was often aligned with the somatic mutation. In rare medical cases, some MPNs, such as ‘primary myelofibrosis’, may accelerate and result in acute myeloid leukaemia. MPNs develop very slowly, so there is a chance that people have them for years prior to noticing their symptoms. It is also known as myeloproliferative disorders or chronic myeloproliferative neoplasms. Chronic delivers the meaning that a condition is long-term. In rare cases, MPNs turn into more critical diseases.

Are MPNs a form of cancer?

Yes, MPNs are a type of blood cancer. Myeloproliferative neoplasms are not a single disease but a group of blood cancers, as they are characterised by the bone marrow producing too many blood cells. These conditions are known as chronic, as they can progress slowly over time. In addition, people having MPNs have excessive blood cell production in their body as the bone marrow produces too many of one or more types of blood cells, resulting in an imbalance in the blood. It can be seen that

Is myeloproliferative disorder curable?

Myeloproliferative disorders are generally not curable, but multiple medical treatments are available to manage the symptoms and prevent complications. Some patients may undergo stem cell transplantation options like ‘allogeneic stem cell transplantation’ that can be curative; however, it is not the option for all because they may not be able to go through the strenuous stem cell transplantation process. Further, the goal of the medical treatment is usually to normalise the blood cell counts and decrease the risk of critical conditions such as blood clots or progression of it to more aggressive forms of cancer. While a cure is not always possible, effective medical treatments exist to manage MPNs, which helps to improve quality of life and extend lifespan with adequate medical treatment. There are some that can result in remissions. Treatment can vary based upon the types of MPNs.

• Primary myelofibrosis: Your doctor may go for monitoring your medical condition very closely if you are not experiencing expected symptoms. They may give drugs or include procedures to treat anaemia.
• Polycythaemia vera: The most common procedure to treat polycythaemia vera is phlebotomy. Your healthcare experts will remove blood regularly in order to reduce blood volume and remove excess blood cells.
• Chronic eosinophilic leukaemia: Your healthcare experts will work to decrease your eosinophil levels through immunotherapy, corticosteroids, or chemotherapy.
• Chronic myelogenous leukaemia: Targeted therapy is the most common treatment, as it prevents cells from multiplying in an uncontrolled or abnormal way. Other treatments are also available, such as chemotherapy, immunotherapy, radiation therapy and stem cell transplants.

What is the cause of myeloproliferative disease?

All myeloproliferative neoplasms are acquired genetic disorders, and it means that you cannot inherit these disorders from your biological parents. These diseases occur when genes that are regulating cell growth change or mutate and a patient’s blood cell development goes wrong. The exact cause of myeloproliferative neoplasm disorder is not fully understood, but usually there is a genetic mutation, especially in the JAK2 gene, observed in individuals having MPNs. It is considered a blood cancer that is caused by the changes in the stem cells inside the bone marrow.

According to the medical research advocates, some discoveries related to the genetic mutation that causes myeloproliferative neoplasms are given below:

• Mutation associated with JAK (Janus kinases): Primary myelofibrosis (polycythaemia vera), as well as essential thrombocythemia, is usually induced by the genetic mutations associated with the protein known as JAK2. Because of this mutation, cells are multiplying in an uncontrolled or abnormal way.
• Mutation related to the MPL or CALR gene: Patients with primary myelofibrosis and essential thrombocythemia usually have mutations in their MPLgene or CALRgene.
• Errors in Chromosome: Individuals with chronic ‘myelogenous leukaemia (CML)’ have a particular error involved in their chromosomes. A chromosome is mainly characterised by being a structure that contains genes. With CML, a piece of chromosome exchanges the place with another chromosome and ultimately forms the “Philadelphia chromosome”.

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