Polycythemia medical therapy
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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.
Sumptomatic Treatment in Polycythemia Vera[4]
| Symptoms | Treatment |
|---|---|
| Pruritus | H1 and H2 blocking antihistamines (diphenhydramine [Benadryl], cyproheptadine [Periactin], hydroxyzine [Atarax, Vistaril], fexofenadine [Allegra], terfenadine [Seldane]) Paroxetine (Paxil) Oatmeal or starch baths (in lukewarm water) Recombinant interferon alfa-2b (intronA) |
| Erythromelalgia | Aspirin, 50 to 100 mg daily Myelosuppressive agents |
Myelosuppressive Agents for the Treatment of Polycythemia Vera[4]
| Agent | Class | Common side effects | Uncommon side effects | Percautions |
|---|---|---|---|---|
| Hydroxyurea (Hydrea) | Antimetabolite | Anemia, neutropenia, oral ulcers, skin ulcers, hyperpigmentation, nail changes | Leg ulcers, nausea, diarrhea, fever, elevated liver function test results | Renal disease |
| Recombinant interferon alfa-2b (Intron A) | Myelosuppressive | Influenza-like symptoms, fatigue, anorexia, weight loss, alopecia, headache, nausea, insomnia, body pain | Confusion, depression, autoimmunity, hyperlipidemia | Psychiatric disease, cardiovascular disease |
| Radioactive phosphorus | Radiopharmaceutica | Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment | Diarrhea, fever, nausea, emesis | — |
| Busulfan (Myleran) | Alkylating agent | Pancytopenia, hyperpigmentation, ovarian suppression | Pulmonary fibrosis, leukemia, seizure, hepatic veno-occlusion | Seizure disorder |
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.
- ↑ 4.0 4.1 Stuart BJ, Viera AJ (2004). "Polycythemia vera". Am Fam Physician. 69 (9): 2139–44. PMID 15152961.