Polycythemia medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
*Phlebotomy is | *Phlebotomy: the backbone of therapy, treatment with phlebotomy alone associated with a longer median survival compared to the use of chlorambucil or radioactive phosphorous based on a trial conducted by PV study group. Repeated phlebotomies help in cytoreduction and reduce hyper-viscosity in addition to induce a state of iron-deficiency which can help retard red-cell proliferation. Weekly sessions are conducted, by remove 500ml of blood until a target hematocrit of under 45% is obtained. This can lower rates of cardiovascular deaths and major thrombotic episodes in patients kept under this threshold based on a trial conducted in Italy. For secondary polycythemias, phlebotomy is usually reserved for the following conditions: | ||
**Chronic lung diseases | |||
**Cyanotic heart diseases | |||
**Post-renal transplant patients with hypertension and erythrocytosis, not responding to optimal doses of angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARB) <ref name="pmid24120605">{{cite journal| author=Assi TB, Baz E| title=Current applications of therapeutic phlebotomy. | journal=Blood Transfus | year= 2014 | volume= 12 Suppl 1 | issue= | pages= s75-83 | pmid=24120605 | doi=10.2450/2013.0299-12 | pmc=3934278 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24120605 }} </ref> <ref name="pmid30252337">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume= | issue= | pages= | pmid=30252337 | doi= | pmc= | url= }} </ref> | |||
*Medicines that may be used include:<ref name="pmid31151982">{{cite journal| author=Spivak JL| title=How I treat polycythemia vera. | journal=Blood | year= 2019 | volume= 134 | issue= 4 | pages= 341-352 | pmid=31151982 | doi=10.1182/blood.2018834044 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31151982 }} </ref> | *Medicines that may be used include:<ref name="pmid31151982">{{cite journal| author=Spivak JL| title=How I treat polycythemia vera. | journal=Blood | year= 2019 | volume= 134 | issue= 4 | pages= 341-352 | pmid=31151982 | doi=10.1182/blood.2018834044 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31151982 }} </ref> | ||
**Hydroxyurea: it used to reduce red blood cells counts and other blood cells if high. It also used to prevent thrombosis in patients >60 years of age with chronic PV. | **Hydroxyurea: it used to reduce red blood cells counts and other blood cells if high. It also used to prevent thrombosis in patients >60 years of age with chronic PV. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2]
Overview
- Phlebotomy: the backbone of therapy, treatment with phlebotomy alone associated with a longer median survival compared to the use of chlorambucil or radioactive phosphorous based on a trial conducted by PV study group. Repeated phlebotomies help in cytoreduction and reduce hyper-viscosity in addition to induce a state of iron-deficiency which can help retard red-cell proliferation. Weekly sessions are conducted, by remove 500ml of blood until a target hematocrit of under 45% is obtained. This can lower rates of cardiovascular deaths and major thrombotic episodes in patients kept under this threshold based on a trial conducted in Italy. For secondary polycythemias, phlebotomy is usually reserved for the following conditions:
- Medicines that may be used include:[3]
- Hydroxyurea: it used to reduce red blood cells counts and other blood cells if high. It also used to prevent thrombosis in patients >60 years of age with chronic PV.
- Interferon: reduce blood cell counts.PegIFN can be used to reduce established splenomegaly but not usually to normal size.
- Anagrelide: used to treat thrombocytosis.
- Ruxolitinib (JAK1/2 inhibitor): proved to be effective in PPMF and chronic-phase PV; provided durable symptom relief, blood count control, and reduction in splenomegaly; and was superior to hydroxyurea.
- Aspirin: used to prevent thrombosis.
- Ultraviolet-B light therapy: reduce the itchiness.
References
- ↑ Assi TB, Baz E (2014). "Current applications of therapeutic phlebotomy". Blood Transfus. 12 Suppl 1: s75–83. doi:10.2450/2013.0299-12. PMC 3934278. PMID 24120605.
- ↑ "StatPearls". 2020. PMID 30252337.
- ↑ Spivak JL (2019). "How I treat polycythemia vera". Blood. 134 (4): 341–352. doi:10.1182/blood.2018834044. PMID 31151982.