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Post polycythemia vera myelofibrosis is a condition in which blood disorderliness develops due to polycythemia vera . Polycythemia vera is a disorder represented by unnatural production of red and white blood cells along with platelets . It progresses to myelofibrosis which is a condition in which lineage cells enumerate keep down due to the scarring ofbone marrow . Post polycythemia vera myelofibrosis is a serious condition that lasts long and cause severe anemia and infections . It may also progress further conduct toblood canceri.e . leukemia . Its symptoms includenight sudate , weightiness loss , fever , weariness , andweakness .
What Is Post Polycythemia Vera Myelofibrosis?
Post polycythemia vera myelofibrosis is a shape that develops when myelofibrosis evolves from polycythemia vera due to genetic mutations . This status is a rare disorder that is long lasting furnish life - peril aftermath . The accurate pathological consequence materialize in this disease is not known . It appear mostly in old grownup who are above the old age of 60 eld . It leads to overenlargement of the spleen .
Polycythemia vera ( PV ) is a term that leads to excess output of bloodline cell that can cause coagulation , stroke or a spunk attack . This disease appears easy that worsens with time . It is unremarkably detected when a person is 60 days old or above but it can develop at any eld . It causes symptom of anaemia with gross blowup of the spleen .
Myelofibrosis is a bone heart and soul disorder that strangle the power of bone marrow to produce origin cellular telephone . This happens due to the fibrosis or scarring of the osseous tissue gist that does not permit it to function usually . It abridge the enumeration of stock electric cell direct to the symptom of anemia , leech disorderliness and many more.(1 )
When polycythemia vera progresses to malignant disease , then it causes a precondition namely carry polycythemia vera myelofibrosis . It is a rarefied disease that causes life-threatening anemia and other life - jeopardize consequence . This upset develops slowly over a pair of many years . Polycythemia vera leads to abnormal production of too many ancestry cells that put special work load on the short temper 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 have a drastic decline in the number of blood cellular telephone below normal . This cause myelofibrosis . The endangerment factors that are connect with the progress of polycythemia vera into myelofibrosis are-
All these changes materialize due to mutant in JAK2 gene . The survival charge per unit of the patient role ranges from 5 - 8 years after diagnosing .
The symptoms of spot polycythemia vera myelofibrosis include Nox travail , fatigue , failing , unexplained fever , and weight unit loss . The body temperature is greater than 37.5 degree Celsius . The weighting is lost by more than 10 % in the previous 6 months . The symptoms of genus Anemia are severe and it exert too much workload on lien that make enlargement of the short temper .
put up polycythemia vera myelofibrosis is diagnosed by the medical history of the affected role that always have polycythemia vera and by blood trial run , pearl marrow biopsy and other tests that find out quick temper blowup .
send polycythemia vera myelofibrosis is managed with radiotherapy , chemotherapy or surgery that either deoxidize the blown-up short temper or remove the spleen . Hydroxyurea , androgen , glucocorticoid , erythropoietin , and ruxolitinib are dictate to control the symptoms . Pomalidomide is the best medicinal drug for this disease .
The median natural selection pace for this disease is 5 - 8 days after the diagnosis .
Conclusion
Post polycythemia vera myelofibrosis is a rare inveterate disease in which polycythemia vera may get myelofibrosis . It is a liveliness - imperil condition that causes knockout anaemia and contagion in the trunk . It leads to extensive fibrosis of bone marrow and enlargement of the spleen . The survival charge per unit of the patient after the diagnosing of this consideration is only 5 - 8 years .
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