Polycythemia medical therapy: Difference between revisions

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{{Polycythemia}}
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{{CMG}} {{AE}}{{Debduti}}  
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==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:
**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 considered as a second-line therapy, it showed lower rates of [[thrombosis]] compared to [[phlebotomy]] alone based on a study by the [[PVSG]].
**Interferon: reduce blood cell counts.PegIFN can be used to reduce established splenomegaly but not usually to normal size.
Indications for use include poor [[venous]] access, high [[phlebotomy]] requirement, when [[phlebotomy]] is [[contraindicated]] or not possible, severe [[thrombocytosis]], and unmanageable [[pruritus]].
**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.  
*'''[[Interferon]]''': reduce [[blood cell]] counts. [[Peg interferon]] can be used to reduce established [[splenomegaly]] but not to normal size.
**Aspirin: used to prevent thrombosis.
*'''[[Anagrelide]]''': used to treat [[thrombocytosis]].
*Ultraviolet-B light therapy: reduce the itchiness.
*'''[[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.<ref name="pmid22375970">{{cite journal| author=Harrison C, Kiladjian JJ, Al-Ali HK, Gisslinger H, Waltzman R, Stalbovskaya V | display-authors=etal| title=JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. | journal=N Engl J Med | year= 2012 | volume= 366 | issue= 9 | pages= 787-98 | pmid=22375970 | doi=10.1056/NEJMoa1110556 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22375970  }} </ref>
*'''[[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.<ref name="pmid15897170">{{cite journal| author=Madkan VK, Bandow GD, Koo JY| title=Resolution of pruritus secondary to polycythemia vera in a patient treated with narrow-band ultraviolet B phototherapy. | journal=J Dermatolog Treat | year= 2005 | volume= 16 | issue= 1 | pages= 56-7 | pmid=15897170 | doi=10.1080/09546630410024529 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15897170  }} </ref>
 
 
'''Sumptomatic Treatment in Polycythemia Vera'''<ref name="pmid15152961">{{cite journal| author=Stuart BJ, Viera AJ| title=Polycythemia vera. | journal=Am Fam Physician | year= 2004 | volume= 69 | issue= 9 | pages= 2139-44 | pmid=15152961 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15152961  }} </ref>
{| style="border: 2px solid #4479BA; align="left"
! style="width: 200px; background: #4479BA;" | {{fontcolor|#FFF|Symptoms}}
! style="width: 300px; background: #4479BA;" | {{fontcolor|#FFF|Treatment}}
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pruritus
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | H1 and H2 blocking antihistamines (diphenhydramine [Benadryl], cyproheptadine [Periactin], hydroxyzine [Atarax, Vistaril], fexofenadine [Allegra], terfenadine [Seldane])<br>Paroxetine (Paxil)<br>Oatmeal or starch baths (in lukewarm water)<br>Recombinant interferon alfa-2b (intronA)
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Erythromelalgia
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Aspirin, 50 to 100 mg daily<br>Myelosuppressive agents
|-
|}
{{clear}}
'''Myelosuppressive Agents for the Treatment of Polycythemia Vera'''<ref name="pmid15152961">{{cite journal| author=Stuart BJ, Viera AJ| title=Polycythemia vera. | journal=Am Fam Physician | year= 2004 | volume= 69 | issue= 9 | pages= 2139-44 | pmid=15152961 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15152961  }} </ref>
{| style="border: 2px solid #4479BA; align="left"
! style="width: 200px; background: #4479BA;" | {{fontcolor|#FFF|Agent}}
! style="width: 300px; background: #4479BA;" | {{fontcolor|#FFF|Class}}
! style="width: 400px; background: #4479BA;" | {{fontcolor|#FFF|Common side effects}}
! style="width: 500px; background: #4479BA;" | {{fontcolor|#FFF|Uncommon side effects}}
! style="width: 600px; background: #4479BA;" | {{fontcolor|#FFF|Percautions}}
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Hydroxyurea (Hydrea)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Antimetabolite
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Anemia, neutropenia, oral ulcers, skin ulcers, hyperpigmentation, nail changes
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Leg ulcers, nausea, diarrhea, fever, elevated liver function test results
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Renal disease
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Recombinant interferon alfa-2b (Intron A)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Myelosuppressive
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Influenza-like symptoms, fatigue, anorexia, weight loss, alopecia, headache, nausea, insomnia, body pain
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Confusion, depression, autoimmunity, hyperlipidemia
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Psychiatric disease, cardiovascular disease
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Radioactive phosphorus
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Radiopharmaceutica
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Diarrhea, fever, nausea, emesis
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | —
|-
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Busulfan (Myleran)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Alkylating agent
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pancytopenia, hyperpigmentation, ovarian suppression
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pulmonary fibrosis, leukemia, seizure, hepatic veno-occlusion
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Seizure disorder
|-
|}
{{clear}}


==References==
==References==

Latest revision as of 18:24, 24 January 2021

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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]Zaida Obeidat, M.D.


Indications for use include poor venous access, high phlebotomy requirement, when phlebotomy is contraindicated or not possible, severe thrombocytosis, and unmanageable pruritus.

  • 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

  1. ↑ Spivak JL (2019). "How I treat polycythemia vera". Blood. 134 (4): 341–352. doi:10.1182/blood.2018834044. PMID 31151982.
  2. ↑ 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.
  3. ↑ 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. ↑ 4.0 4.1 Stuart BJ, Viera AJ (2004). "Polycythemia vera". Am Fam Physician. 69 (9): 2139–44. PMID 15152961.

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