Polycythemia vera (PV) is a form of blood cancer characterized by an excess RBC synthesis and increased RBC mass.
It causes the bone marrow to produce excessive red blood cells. These extra cells thicken the blood, prolonging its flow and causing complications like leukemia and blood clots.
Epidemiology
PV affects individuals of any ethnicity and gender though males are slightly more likely to be affected than women. It can affect people of various ages, although the typical age of diagnosis is 60.
PV affects 1.6 individuals per million in the United States. There is a lower incidence in Japan than in Europe or the United States.
Anatomy
Pathophysiology
Patients with PV have normal and abnormal clonal stem cells in their bone marrow, inhibiting healthy stem cells’ development and maturation. When the neoplastic growth is not under control, it causes panmyelosis.
PV is most likely caused by signaling abnormalities brought on by JAK2 kinase mutation. The JAK2 gene’s valine to phenylalanine substitution, or JAK2V617F, results in constitutively activated cytokine receptors.
This mutation is seen in over 90% of PV patients, 50-60% of myelofibrosis patients, and 50% of thrombocythemia patients. This process increases the synthesis of red blood cells and platelets, as well as the complications of thrombosis and bleeding.
Etiology
The disease process is triggered by neoplastic growth. A signaling deficiency causes an abnormal reaction to growth stimuli, and the abnormal clonal line disrupts normal lineage proliferation.
In 90% of instances, the Janus kinase-2 (JAK2) gene is altered, which is essential in intracellular signaling. The cytogenetic finding indicates an abnormal karyotype in around 34% of PV patients in hematopoietic progenitor cells.
At the time of diagnosis, 20% of patients have cytogenetic abnormalities, which increases to 80% after more than ten years of follow-up therapy.
Genetics
Prognostic Factors
Untreated polycythemia vera has a median survival span of 18 months, but individuals with treatment intervention have a life expectancy of 14 years and about 24 years if the patient is younger than 60.
According to a study, the five-year survival rate was 79.5%; however, patients are at significant risk of developing other primary malignancies and leukemic transformation, which may impair long-term survival.
ruxolitinib is indicated to treat polycythemia vera in patients who are intolerant to hydroxyurea and show inadequate response to the same
A dose of 10 mg orally twice daily is specified initially
hydroxyurea not in use currently
Initial dosage: 100 mcg Subcutaneous every two Weeks
Increase dosage by 50 mcg every two weeks (but no more than 500 mcg per dose) till haematological parameters (platelets [Plt] <400x 109/L, haematocrit [Hct] <45%], leukocytes <10x 109/L) are stabilized.
transition from hydroxyurea
The initial dosage is 50 mcg subcutaneously every two weeks in combination with hydroxyurea
When haematological parameters (Plt 400 x 109/L, HCT 45%, and leukocytes 10 x 109/L) stabilize, the dose should be increased by 50 mcg every two weeks (but not more than 500 mcg/dose).
Weeks 3 to 12: Decrease the total biweekly hydroxyurea dosage by 20–40% every two weeks.
Week 13: stop using hydroxyurea.
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References
https://www.ncbi.nlm.nih.gov/books/NBK557660/
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Polycythemia vera (PV) is a form of blood cancer characterized by an excess RBC synthesis and increased RBC mass.
It causes the bone marrow to produce excessive red blood cells. These extra cells thicken the blood, prolonging its flow and causing complications like leukemia and blood clots.
PV affects individuals of any ethnicity and gender though males are slightly more likely to be affected than women. It can affect people of various ages, although the typical age of diagnosis is 60.
PV affects 1.6 individuals per million in the United States. There is a lower incidence in Japan than in Europe or the United States.
Patients with PV have normal and abnormal clonal stem cells in their bone marrow, inhibiting healthy stem cells’ development and maturation. When the neoplastic growth is not under control, it causes panmyelosis.
PV is most likely caused by signaling abnormalities brought on by JAK2 kinase mutation. The JAK2 gene’s valine to phenylalanine substitution, or JAK2V617F, results in constitutively activated cytokine receptors.
This mutation is seen in over 90% of PV patients, 50-60% of myelofibrosis patients, and 50% of thrombocythemia patients. This process increases the synthesis of red blood cells and platelets, as well as the complications of thrombosis and bleeding.
The disease process is triggered by neoplastic growth. A signaling deficiency causes an abnormal reaction to growth stimuli, and the abnormal clonal line disrupts normal lineage proliferation.
In 90% of instances, the Janus kinase-2 (JAK2) gene is altered, which is essential in intracellular signaling. The cytogenetic finding indicates an abnormal karyotype in around 34% of PV patients in hematopoietic progenitor cells.
At the time of diagnosis, 20% of patients have cytogenetic abnormalities, which increases to 80% after more than ten years of follow-up therapy.
Untreated polycythemia vera has a median survival span of 18 months, but individuals with treatment intervention have a life expectancy of 14 years and about 24 years if the patient is younger than 60.
According to a study, the five-year survival rate was 79.5%; however, patients are at significant risk of developing other primary malignancies and leukemic transformation, which may impair long-term survival.
ruxolitinib is indicated to treat polycythemia vera in patients who are intolerant to hydroxyurea and show inadequate response to the same
A dose of 10 mg orally twice daily is specified initially
hydroxyurea not in use currently
Initial dosage: 100 mcg Subcutaneous every two Weeks
Increase dosage by 50 mcg every two weeks (but no more than 500 mcg per dose) till haematological parameters (platelets [Plt] <400x 109/L, haematocrit [Hct] <45%], leukocytes <10x 109/L) are stabilized.
transition from hydroxyurea
The initial dosage is 50 mcg subcutaneously every two weeks in combination with hydroxyurea
When haematological parameters (Plt 400 x 109/L, HCT 45%, and leukocytes 10 x 109/L) stabilize, the dose should be increased by 50 mcg every two weeks (but not more than 500 mcg/dose).
Weeks 3 to 12: Decrease the total biweekly hydroxyurea dosage by 20–40% every two weeks.
Week 13: stop using hydroxyurea.
https://www.ncbi.nlm.nih.gov/books/NBK557660/
medtigo
Polycythemia vera
Updated :
September 20, 2022
Polycythemia vera (PV) is a form of blood cancer characterized by an excess RBC synthesis and increased RBC mass.
It causes the bone marrow to produce excessive red blood cells. These extra cells thicken the blood, prolonging its flow and causing complications like leukemia and blood clots.
PV affects individuals of any ethnicity and gender though males are slightly more likely to be affected than women. It can affect people of various ages, although the typical age of diagnosis is 60.
PV affects 1.6 individuals per million in the United States. There is a lower incidence in Japan than in Europe or the United States.
Patients with PV have normal and abnormal clonal stem cells in their bone marrow, inhibiting healthy stem cells’ development and maturation. When the neoplastic growth is not under control, it causes panmyelosis.
PV is most likely caused by signaling abnormalities brought on by JAK2 kinase mutation. The JAK2 gene’s valine to phenylalanine substitution, or JAK2V617F, results in constitutively activated cytokine receptors.
This mutation is seen in over 90% of PV patients, 50-60% of myelofibrosis patients, and 50% of thrombocythemia patients. This process increases the synthesis of red blood cells and platelets, as well as the complications of thrombosis and bleeding.
The disease process is triggered by neoplastic growth. A signaling deficiency causes an abnormal reaction to growth stimuli, and the abnormal clonal line disrupts normal lineage proliferation.
In 90% of instances, the Janus kinase-2 (JAK2) gene is altered, which is essential in intracellular signaling. The cytogenetic finding indicates an abnormal karyotype in around 34% of PV patients in hematopoietic progenitor cells.
At the time of diagnosis, 20% of patients have cytogenetic abnormalities, which increases to 80% after more than ten years of follow-up therapy.
Untreated polycythemia vera has a median survival span of 18 months, but individuals with treatment intervention have a life expectancy of 14 years and about 24 years if the patient is younger than 60.
According to a study, the five-year survival rate was 79.5%; however, patients are at significant risk of developing other primary malignancies and leukemic transformation, which may impair long-term survival.
https://www.ncbi.nlm.nih.gov/books/NBK557660/
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