Polycythemia medical therapy: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
 
(13 intermediate revisions by the same user not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Polycythemia}}
{{Polycythemia}}
{{CMG}} {{AE}}{{Debduti}}  
{{CMG}} {{AE}}{{Debduti}}{{ZO}}  


==Overview==
*Phlebotomy is used to decrease blood thickness.
*Medicines that may be used include:
**Hydroxyurea: reduce the number of red blood cells made by the bone marrow. This drug may be used when the numbers of other blood cell types are also high.
**Interferon: lower blood counts.
**Anagrelide: lower platelet counts.
**Ruxolitinib (Jakafi): reduce the number of red blood cells and reduce an enlarged spleen. This drug is prescribed when hydroxyurea and other treatments have failed.
**Aspirin: reduce the risk of blood clots.


*Ultraviolet-B light therapy: reduce the itchiness.
*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 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]].


==Medical Therapy==
*'''[[Interferon]]''': reduce [[blood cell]] counts. [[Peg interferon]] can be used to reduce established [[splenomegaly]] but not to normal size.
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].  
*'''[[Anagrelide]]''': used to treat [[thrombocytosis]].
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*'''[[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>
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*'''[[Aspirin]]''': indicated at low dose to prevent [[thrombosis]], when there is [[insufficient]] control of [[microvascular]] [[symptoms]] or other [[cardiovascular]] risk factors exist.
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
*'''[[Hypouricemic]] Agents ([[Allopurinol]] and [[febuxostat]])''': required if there is significant [[hyperuricemia]].
===Disease Name===


* '''1 Stage 1 - Name of stage'''
*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>
** 1.1 '''Specific Organ system involved 1'''
*** 1.1.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''  
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2 '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
 
** 2.1 '''Specific Organ system involved 1 '''
'''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>
**: '''Note (1):'''
{| style="border: 2px solid #4479BA; align="left"
**: '''Note (2)''':  
! style="width: 200px; background: #4479BA;" | {{fontcolor|#FFF|Symptoms}}
**: '''Note (3):'''
! style="width: 300px; background: #4479BA;" | {{fontcolor|#FFF|Treatment}}
*** 2.1.1 '''Adult'''
|-
**** Parenteral regimen
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pruritus
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
| 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)
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
|-
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Erythromelalgia
**** Oral regimen
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Aspirin, 50 to 100 mg daily<br>Myelosuppressive agents
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
|-
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
|}
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
{{clear}}
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
'''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>
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
{| style="border: 2px solid #4479BA; align="left"
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
! style="width: 200px; background: #4479BA;" | {{fontcolor|#FFF|Agent}}
*** 2.1.2 '''Pediatric'''
! style="width: 300px; background: #4479BA;" | {{fontcolor|#FFF|Class}}
**** Parenteral regimen
! style="width: 400px; background: #4479BA;" | {{fontcolor|#FFF|Common side effects}}
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
! style="width: 500px; background: #4479BA;" | {{fontcolor|#FFF|Uncommon side effects}}
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
! style="width: 600px; background: #4479BA;" | {{fontcolor|#FFF|Percautions}}
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
|-
**** Oral regimen
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Hydroxyurea (Hydrea)
***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Antimetabolite
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Anemia, neutropenia, oral ulcers, skin ulcers, hyperpigmentation, nail changes
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Leg ulcers, nausea, diarrhea, fever, elevated liver function test results
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Renal disease
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
|-
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Recombinant interferon alfa-2b (Intron A)
** 2.2 '<nowiki/>'''''Other Organ system involved 2''''''
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Myelosuppressive
**: '''Note (1):'''
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Influenza-like symptoms, fatigue, anorexia, weight loss, alopecia, headache, nausea, insomnia, body pain
**: '''Note (2)''':  
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Confusion, depression, autoimmunity, hyperlipidemia
**: '''Note (3):'''
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Psychiatric disease, cardiovascular disease
*** 2.2.1 '''Adult'''
|-
**** Parenteral regimen
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Radioactive phosphorus
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Radiopharmaceutica
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Diarrhea, fever, nausea, emesis
**** Oral regimen
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | —
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
|-
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Busulfan (Myleran)
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Alkylating agent
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pancytopenia, hyperpigmentation, ovarian suppression
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Pulmonary fibrosis, leukemia, seizure, hepatic veno-occlusion
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
| style="padding: 0 5px; background: #F5F5F5; text-align: left;" | Seizure disorder
*** 2.2.2 '''Pediatric'''
|-
**** Parenteral regimen
|}
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
{{clear}}
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)


==References==
==References==

Latest revision as of 18:24, 24 January 2021

Polycythemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Polycythemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Polycythemia medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Polycythemia medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Polycythemia medical therapy

CDC on Polycythemia medical therapy

Polycythemia medical therapy in the news

Blogs on Polycythemia medical therapy

Directions to Hospitals Treating Polycythemia

Risk calculators and risk factors for Polycythemia medical therapy

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.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Polycythemia_medical_therapy&oldid=1685467"