Post polycythemia vera myelofibrosis is a condition in which blood disorder develops due to polycythemia vera. Polycythemia vera is a disorder represented by abnormal production of red and white blood cells along with platelets. It progresses to myelofibrosis which is a condition in which blood cells count reduce due to the scarring of bone marrow. Post polycythemia vera myelofibrosis is a serious condition that lasts long and cause severe anemia and infections. It may also progress further leading to blood cancer i.e. leukemia. Its symptoms include night sweats, weight loss, fever, fatigue, and weakness.
What Is Post Polycythemia Vera Myelofibrosis?
Post polycythemia vera myelofibrosis is a condition that develops when myelofibrosis evolves from polycythemia vera due to genetic mutations. This condition is a rare disorder that is long lasting rendering life-threatening consequences. The exact pathological events happening in this disease is not known. It appears mostly in old adults who are above the age of 60 years. It leads to over enlargement of the spleen.
Polycythemia vera (PV) is a condition that leads to excess production of blood cells that can cause clotting, stroke or a heart attack. This disease appears slowly that worsens with time. It is usually detected when a person is 60 years old or above but it can develop at any age. It causes symptoms of anemia with gross enlargement of the spleen.
Myelofibrosis is a bone marrow disorder that hampers the ability of bone marrow to produce blood cells. This happens due to the fibrosis or scarring of the bone marrow that does not allow it to function normally. It reduces the count of blood cells leading to the symptoms of anemia, bleeding disorders and many more. (1)
When polycythemia vera progresses to malignant disease, then it causes a condition namely post polycythemia vera myelofibrosis. It is a rare disease that causes severe anemia and other life-threatening consequences. This disorder develops slowly over a span of many years. Polycythemia vera leads to abnormal production of too many blood cells that put extra workload on the spleen and bone marrow. It leads to extensive fibrosis of the bone marrow over time. The extensive fibrosis is the hallmark of post polycythemia vera myelofibrosis. It thereby reduces the production of blood cells causing a drastic decline in the number of blood cells below normal. This causes myelofibrosis. The risk factors that are linked with the progress of polycythemia vera into myelofibrosis are-
- Age above 60 years or above
- Increased white blood cell count
- High platelet count
- Fibrosis of bone marrow
- Enlargement of spleen (2)
All these changes happen due to mutations in JAK2 genes. The survival rate of the patient ranges from 5-8 years after diagnosis.
The symptoms of post polycythemia vera myelofibrosis include night sweat, fatigue, weakness, unexplained fever, and weight loss. The body temperature is greater than 37.5 degree Celsius. The weight is lost by more than 10 % in the previous 6 months. The symptoms of anemia are severe and it exerts too much workload on spleen that causes enlargement of the spleen.
Post polycythemia vera myelofibrosis is diagnosed by the medical history of the patient that always have polycythemia vera and by blood tests, bone marrow biopsy and other tests that find out spleen enlargement.
Post polycythemia vera myelofibrosis is managed with radiotherapy, chemotherapy or surgery that either reduces the enlarged spleen or removes the spleen. Hydroxyurea, androgens, glucocorticoids, erythropoietin, and ruxolitinib are prescribed to control the symptoms. Pomalidomide is the best medicine for this disease.
The median survival rate for this disease is 5-8 years after the diagnosis.
Conclusion
Post polycythemia vera myelofibrosis is a rare chronic disease in which polycythemia vera may cause myelofibrosis. It is a life-threatening condition that causes severe anemia and infections in the body. It leads to extensive fibrosis of bone marrow and enlargement of the spleen. The survival rate of the patient after the diagnosis of this condition is only 5-8 years.
