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
- Medicines that may be used include:[1]
- Hydroxyurea: it considered as a second-line therapy, it showed lower rates of thrombosis compared to phlebotomy alone based on a study by the PVSG.
Indications for use include poor venous access, high phlebotomy requirement, when phlebotomy is contraindicated or not possible, severe thrombocytosis, and unmanageable pruritus.
- Interferon: reduce blood cell counts. Peg interferon can be used to reduce established splenomegaly but not to normal size.
- Anagrelide: used to treat thrombocytosis.
- Ruxolitinib (JAK1/2 inhibitor): it used when patients are intolerant or unresponsive to hydroxyurea
-, proved to be effective in PPMF and chronic-phase PV; provided a durable relief in symptoms, blood count control, significant reduction in splenomegaly, and better survival based on COMFORT trials.[2]
- Aspirin: indicated at low dose to prevent thrombosis, when there is insufficient control of microvascular symptoms or other cardiovascular risk factors exist.
- Hypouricemic Agents (Allopurinol and febuxostat): required if there is significant hyperuricemia.
- Management of pruritus: antihistamines and selective serotonin reuptake inhibitors (SSRIs) are usually used to relief the symptoms, narrow band ultraviolet-B phototherapy can also be used to treat pruritus.[3]
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
- ↑ Spivak JL (2019). "How I treat polycythemia vera". Blood. 134 (4): 341–352. doi:10.1182/blood.2018834044. PMID 31151982.
- ↑ Harrison C, Kiladjian JJ, Al-Ali HK, Gisslinger H, Waltzman R, Stalbovskaya V; et al. (2012). "JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis". N Engl J Med. 366 (9): 787–98. doi:10.1056/NEJMoa1110556. PMID 22375970.
- ↑ Madkan VK, Bandow GD, Koo JY (2005). "Resolution of pruritus secondary to polycythemia vera in a patient treated with narrow-band ultraviolet B phototherapy". J Dermatolog Treat. 16 (1): 56–7. doi:10.1080/09546630410024529. PMID 15897170.
- ↑ 4.0 4.1 Stuart BJ, Viera AJ (2004). "Polycythemia vera". Am Fam Physician. 69 (9): 2139–44. PMID 15152961.