About MPD (Myeloproliferative Disease)

Myeloproliferative diseases is a term used for a cluster of illnesses. These diseases are when the bone marrow creates more red/white blood cells or platelets then the body needs. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis together comprise the majority of myeloproliferative diseases.
 
The total number of people living with myeloproliferative diseases (prevalence rate) in the US is estimated to be 29,5709. This estimation is based on 2010 information.  
In the US Medicare population, 53.2% of patients with essential thrombocythemia (ET) survive after 5 Years. This means out of 100 patients with essential thrombocythemia (ET), about 53 patients have survived the disease for 5 years.  In the US Medicare population, 53.1% of patients with polycythemia vera (PV) survive after 5 Years. This means out of 100 patients with polycythemia vera (PV), about 53 patients have survived the disease for 5 years. In the US Medicare population, 18.2% of patients with myelofibrosis (MF) survive after 5 years. This means out of 100 patients with myelofibrosis (MF), about 53 patients have survived the disease for 5 years.
 
Risk Factors
There are certain risk factors that can increase the chances of having Myeloproliferative Diseases.

• Genomic causes. Myeloproliferative Diseases are not thought to be heritable. However, there is some data to show that some people may be inclined to develop myeloproliferative diseases based on their own personal genetic codes.
• Factors present in the environment.  Exposure to radiation and petrochemicals (like benzene and toluene) have been linked with the development of myeloproliferative diseases.

Diagnosis

• Blood tests.  If your doctor thinks that you have a myeloproliferative disease, s/he may order blood tests that will take a small amount of blood to look at your cells. Your doctor may order more test if they see abnormal initial blood test results.
• Bone marrow biopsy. A small amount of bone marrow is taken from the bone, typically from the hip bone, with a needle. The sample taken from the bone is called a biopsy. In a laboratory, the biopsy is examined for malformations in the cells

Symptoms
There are certain symptoms and signs showing that a patient may have myeloproliferative disease. Patients may have anemia, shortness of breath with activity, loss of strength or tiredness, paleness, reduction in hunger, prolonged bleeding from minor cuts due to low platelet counts, purpura-black/blue pinpoint marks on the skin where bleeding has occurred under the skin, sinus-skin/urinary infections owing to a reduced number of low white blood cells that fight infection.
Treatment Options
Myeloproliferative disease typically cannot be cured. Treatment typically tries to fix the irregular blood tests.  If a patient has no symptoms, with a doctor’s consultation, the patient may decide to take the watchful waiting approach.  This means the patient will not be on treatment but they will get frequent evaluations and undergo diagnostic checks to monitor the progress of their disease.

• Phlebotomy. This technique is used to decrease the number of red blood cells from the body. A large volume of blood is removed to accomplish this.  It can help with the symptoms connected to having a high number of red blood cells.
• Cell-Reducing Medications. Patients who are at higher risk for clotting and who have higher red blood cell counts may require these drugs. The drugs reduce the number of red blood cells that the body makes or limit their release from the bone marrow. Examples include hydroxyurea (Hydrea), Interferon-alpha and Anagrelide (Agrylin).
• Supportive Care. In the advanced stages of the disease or when the bone marrow is not making normal blood cells treatment may include drugs and transfusion. There are several examples of drugs used for supportive care. For example growth factor erythropoietin (EPO) expands the number of red blood cells, growth factor granulocyte colony-stimulating factor (G-CSF) expands the number of white blood cells. Also transfusions can increase the number of red blood cells/platelets.
• Chemotherapy. Chemotherapy can be presented by injection, by oral delivery, or by injection into the spinal fluid.  The drugs help manage irregular blood cells.
• Transplantation. Those with severe/advanced myeloproliferative disease can be treated with a transplant of bone marrow.
• Drugs targeting certain gene aberration.  The drugs stop abnormal proteins linked to those certain gene mutations. Examples of these drugs include imatinib (Gleevec) and ruxolitinib (Jakafi).

 
References:
“Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version.” National Institutes of Health National Cancer Institute, 2019. https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq.
“Prevalence.” MPN Research Foundation, 2019. https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq.
“Survival Patterns in United States (US) Medicare Enrollees with Non-CML Myeloproliferative Neoplasms (MPN).”  Price et al. 2019.
https://journals.plos.org/plosone/article?id=10.1371%2Fjournal.pone.0090299.
“Myeloproliferative Neoplasms” Cancer Network-Oncology, 2019  
https://www.cancernetwork.com/cancer-management/myeloproliferative-neoplasms/page/0/2
“Myeloproliferative Disorders Signs and Symptoms” University of California San
Francisco, 2019  
https://www.ucsfhealth.org/conditions/myeloproliferative_disorders/signs_and_symptoms.html.
“Treatment Options” Seattle Cancer Care Alliance, 2019.  
https://www.seattlecca.org/diseases/myeloproliferative-neoplasms-mpn/treatment-options.

‌Web links about Myeloproliferative Disorders
Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version
https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq
 
Overview of Myeloproliferative Disorders
https://www.merckmanuals.com/home/blood-disorders/myeloproliferative-disorders/overview-of-myeloproliferative-disorders
 
Drugs Approved for Myeloproliferative Neoplasms
https://www.cancer.gov/about-cancer/treatment/drugs/myeloproliferative-neoplasms

Chronic myeloproliferative disorders
https://rarediseases.info.nih.gov/diseases/9319/chronic-myeloproliferative-disorders


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