Myelofibrosis medical therapy: Difference between revisions

	Myelofibrosis Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Myelofibrosis 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 and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Myelofibrosis medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Myelofibrosis medical therapy All Images X-rays Echo and Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Myelofibrosis medical therapy
CDC on Myelofibrosis medical therapy
Myelofibrosis medical therapy in the news
Blogs on Myelofibrosis medical therapy
Directions to Hospitals Treating Myelofibrosis
Risk calculators and risk factors for Myelofibrosis medical therapy

VisualWikitext

Latest revision as of 22:25, 31 January 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]Sujit Routray, M.D. [3]

Overview

Red blood cell transfusion, danazol therapy, or thalidomide are recommended for patients who develop anemia. Ruxolitinib, an inhibitor of Janus kinase 1 (JAK1) and Janus kinase 2 (JAK2), can reduce the splenomegaly and the constitutional symptoms of weight loss, fatigue, and night sweats for patients with Janus kinase 2 (JAK2)-positive or Janus kinase 2 (JAK2)-negative primary myelofibrosis (PMF), post–essential thrombocythemia myelofibrosis, or post–polycythemia vera myelofibrosis. Hydroxyurea, chemotherapy, or radiotherapy are recommended for patients who develop splenomegaly.

Medical Therapy

Treatment for Anemia

Red blood cell transfusion

The profound anemia that develops in this disease usually requires red blood cell transfusion.^[1]^[2]

Glucocorticoids

Glucocorticoids can improve the red blood cell (RBC) survival, which can be markedly decreased in some patients.^[1]^[3]

Androgen therapy

Danazol can be given to stimulate the production of red blood cells (RBCs).
Preferred regimen: 600-800 mg/day till normalization of cell counts and then reduced to 200-400 mg/day

Erythropoietic growth factors

Erythropoietin and darbepoetin can help if patients are not dependent on transfusion.
Preferred regimen: SubQ: 150 to 300 mcg once weekly or 500 mcg once every 2 to 3 weeks

Thalidomide, lenalidomide, or pomalidomide

These may help in improving the red blood cell counts, thus improving anemia and help in the reversal of splenomegaly. However, patients on these drugs require prophylaxis for avoiding thrombosis and careful monitoring for hematologic toxicity.^[1]^[4]
Preferred regimen: Oral: 10 mg once daily

Janus kinase (JAK) Inhibitor Therapy

Ruxolitinib, an inhibitor of Janus kinase 1 (JAK1) and Janus kinase 2 (JAK2), can reduce the splenomegaly and the symptoms of weight loss, fatigue, and night sweats in patients with Janus kinase 2 (JAK2)-positive or Janus kinase 2 (JAK2)-negative primary myelofibrosis, post-essential thrombocythemia myelofibrosis, or post-polycythemia vera myelofibrosis.^[1]^[5]
Ruxolitinib can also be integrated as pretransplantation treatment.^[6]
Ruxolitinib preferred regimen: Oral: Initial dose (based on platelet count, titrate dose thereafter based on efficacy and safety):

Platelets >200,000/mm3: 20 mg twice daily
Platelets 100,000 to 200,000/mm3: 15 mg twice daily
Platelets 50,000 to <100,000/mm3: 5 mg twice daily

Momelotinib is a JAK inhibitor that is currently in clinical trials.
Pacritinib is a JAK inhibitor that is currently in clinical trials.

Treatment for Splenomegaly

JAK2 inhibitor therapy

Ruxolitinib can provide temporary relief.^[1]^[7] This medication is FDA-approved for intermediate- and high-risk myelofibrosis.

Hydroxyurea

Hydroxyurea can reduce the splenomegaly but may have a potential leukemogenic effect.
Preferred regimen: Starting dose of 500 mg/day and then subsequently adjusted to the individual efficacy.

Chemotherapy

Drugs like thalidomide, lenalidomide, or cladribine may also reduce the spleen size, and relieve the pain symptoms.

Radiation therapy

Radiation may be used to reduce the size of the spleen, when splenectomy is contraindicated.

Splenectomy

Splenectomy can also be performed.

Interferon therapy

Peg-interferon alpha 2a and peg-interferon alpha 2b have been shown to reduce spleen size, improve constitutional symptoms, and improve blood counts.

References

↑ ^1.0 ^1.1 ^1.2 ^1.3 ^1.4 Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
↑ Gupta V, Kosiorek HE, Mead A, Klisovic RB, Galvin JP, Berenzon D, Yacoub A, Viswabandya A, Mesa RA, Goldberg J, Price L, Salama ME, Weinberg RS, Rampal R, Farnoud N, Dueck AC, Mascarenhas JO, Hoffman R (September 2018). "Ruxolitinib Therapy Followed by Reduced-Intensity Conditioning for Hematopoietic Cell Transplantation for Myelofibrosis: Myeloproliferative Disorders Research Consortium 114 Study". Biol. Blood Marrow Transplant. doi:10.1016/j.bbmt.2018.09.001. PMID 30205231.
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

Template:WikiDoc Sources

[treatmentoverviewofmyelofibrosis1-1] 1.0 ^1.1 ^1.2 ^1.3 ^1.4 Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016

[2] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[3] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[4] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[5] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[pmid30205231-6] Gupta V, Kosiorek HE, Mead A, Klisovic RB, Galvin JP, Berenzon D, Yacoub A, Viswabandya A, Mesa RA, Goldberg J, Price L, Salama ME, Weinberg RS, Rampal R, Farnoud N, Dueck AC, Mascarenhas JO, Hoffman R (September 2018). "Ruxolitinib Therapy Followed by Reduced-Intensity Conditioning for Hematopoietic Cell Transplantation for Myelofibrosis: Myeloproliferative Disorders Research Consortium 114 Study". Biol. Blood Marrow Transplant. doi:10.1016/j.bbmt.2018.09.001. PMID 30205231.

[7] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[1]

[2]

[3]

[4]

[5]

[6]

[7]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Myelofibrosis}}
-{{CMG}}
+{{CMG}}{{AE}}{{Sab}}{{SR}}
 ==Overview==
+[[Red blood cell]] [[Blood transfusion|transfusion]], [[danazol]] [[therapy]], or [[thalidomide]] are recommended for [[Patient|patients]] who develop [[anemia]]. [[Ruxolitinib]], an [[inhibitor]] of [[Janus kinase 1|Janus kinase 1 (JAK1)]] and [[Janus kinase 2|Janus kinase 2 (JAK2)]], can reduce the [[splenomegaly]] and the constitutional [[Symptom|symptoms]] of [[weight loss]], [[fatigue]], and [[night sweats]] for [[Patient|patients]] with [[Janus kinase 2|Janus kinase 2 (JAK2)]]-positive or [[Janus kinase 2|Janus kinase 2 (JAK2)]]-negative primary myelofibrosis (PMF), post–[[essential thrombocythemia]] myelofibrosis, or post–[[polycythemia vera]] myelofibrosis. [[Hydroxyurea]], [[chemotherapy]], or [[radiotherapy]] are recommended for [[Patient|patients]] who develop [[splenomegaly]].
-==Medical therapy==
+==Medical Therapy==
-Other treatment options are largely supportive, and do not alter the course.<ref name="pmid18185525">{{cite journal |author=Kröger N, Mesa RA |title=Choosing between stem cell therapy and drugs in myelofibrosis |journal=Leukemia |volume=22 |issue=3 |pages=474–86 |year=2008 |month=March |pmid=18185525 |doi=10.1038/sj.leu.2405080 |url=http://dx.doi.org/10.1038/sj.leu.2405080}}</ref> These options may include regular [[folic acid]], [[allopurinol]] or [[blood transfusion]]s. [[Dexamethasone]], alpha-[[interferon]] and [[hydroxycarbamide]] may play a role.
+===Treatment for Anemia===
+'''Red blood cell transfusion'''
+*The profound [[anemia]] that develops in this [[disease]] usually requires [[Blood transfusion|red blood cell transfusion]].<ref name="treatmentoverviewofmyelofibrosis1">Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
+'''Glucocorticoids'''
+*[[Glucocorticoids]] can improve the [[Red blood cell|red blood cell (RBC)]] survival, which can be markedly decreased in some [[Patient|patients]].<ref name="treatmentoverviewofmyelofibrosis1">Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
+'''Androgen therapy'''
+*[[Danazol]] can be given to stimulate the production of [[Red blood cells|red blood cells (RBCs)]].
+*Preferred regimen:  600-800 mg/day till normalization of cell counts and then reduced to 200-400 mg/day
+'''Erythropoietic growth factors'''
+*[[Erythropoietin]] and [[darbepoetin]] can help if [[patients]] are not dependent on [[transfusion]].
+*Preferred regimen: SubQ: 150 to 300 mcg once weekly or 500 mcg once every 2 to 3 weeks
+'''Thalidomide, lenalidomide, or pomalidomide'''
+*These may help in improving the [[red blood cell]] counts, thus improving [[anemia]] and help in the reversal of [[splenomegaly]]. However, [[Patient|patients]] on these drugs require [[prophylaxis]] for avoiding [[thrombosis]] and careful monitoring for [[Hematology|hematologic]] toxicity.<ref name="treatmentoverviewofmyelofibrosis1">Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
+*Preferred regimen: Oral: 10 mg once daily
-[[Lenalidomide]] and [[Thalidomide]] may be used in its treatment, though they can cause [[gout]] and leave the patient susceptible to diseases such as [[pneumonia]].
+===Janus kinase (JAK) Inhibitor Therapy===
+*[[Ruxolitinib]], an [[inhibitor]] of [[Janus kinase 1|Janus kinase 1 (''JAK1)'']] and [[Janus kinase 2|Janus kinase 2 (''JAK2)'']], can reduce the [[splenomegaly]] and the [[Symptom|symptoms]] of [[weight loss]], [[fatigue]], and [[night sweats]] in [[Patient|patients]] with [[Janus kinase 2|Janus kinase 2 (JAK2)]]-positive or [[Janus kinase 2|Janus kinase 2 (JAK2)]]-negative primary myelofibrosis, post-[[essential thrombocythemia]] myelofibrosis, or post-[[polycythemia vera]] myelofibrosis.<ref name="treatmentoverviewofmyelofibrosis1">Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
+*[[Ruxolitinib]] can also be integrated as pretransplantation treatment.<ref name="pmid30205231">{{cite journal |vauthors=Gupta V, Kosiorek HE, Mead A, Klisovic RB, Galvin JP, Berenzon D, Yacoub A, Viswabandya A, Mesa RA, Goldberg J, Price L, Salama ME, Weinberg RS, Rampal R, Farnoud N, Dueck AC, Mascarenhas JO, Hoffman R |title=Ruxolitinib Therapy Followed by Reduced-Intensity Conditioning for Hematopoietic Cell Transplantation for Myelofibrosis: Myeloproliferative Disorders Research Consortium 114 Study |journal=Biol. Blood Marrow Transplant. |volume= |issue= |pages= |date=September 2018 |pmid=30205231 |doi=10.1016/j.bbmt.2018.09.001 |url=}}</ref>
+*Ruxolitinib preferred regimen: Oral: Initial dose (based on platelet count, titrate dose thereafter based on efficacy and safety):
+:*Platelets >200,000/mm3: 20 mg twice daily
+:*Platelets 100,000 to 200,000/mm3: 15 mg twice daily
+:*Platelets 50,000 to <100,000/mm3: 5 mg twice daily
+*[[Momelotinib]] is a JAK inhibitor that is currently in clinical trials.
+*[[Pacritinib]] is a JAK inhibitor that is currently in clinical trials.
-Frequent [[blood transfusions]] may also be required.
+===Treatment for Splenomegaly===
+'''''JAK2'' inhibitor therapy'''
-The patient with the myelofibrosis should be tested for [[Tuberculosis]] even if the patient doesn't manifest the symptoms of TB. There is strong evidence of myelofibrois improvement with the antituberculous treatment.
+*[[Ruxolitinib]] can provide temporary relief.<ref name="treatmentoverviewofmyelofibrosis1">Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref> This medication is FDA-approved for intermediate- and high-risk myelofibrosis.
+'''Hydroxyurea'''
+*[[Hydroxyurea]] can reduce the [[splenomegaly]] but may have a potential leukemogenic effect.
+*Preferred regimen: Starting dose of 500 mg/day and then subsequently adjusted to the individual efficacy.
+'''Chemotherapy'''
+*Drugs like [[thalidomide]], [[lenalidomide]], or [[cladribine]] may also reduce the [[spleen]] size, and relieve the [[pain]] [[Symptom|symptoms]].
+'''Radiation therapy'''
+*[[Radiation]] may be used to reduce the size of the [[spleen]], when [[splenectomy]] is contraindicated.
+'''Splenectomy'''
+*[[Splenectomy]] can also be performed.
+'''Interferon therapy'''
+*Peg-interferon alpha 2a and peg-interferon alpha 2b have been shown to reduce spleen size, improve constitutional symptoms, and improve blood counts.
 ==References==
@@ Line 19: / Line 52: @@
 [[Category:Disease]]
 [[Category:Types of cancer]]
+[[Category:Hematology]]
 [[Category:Oncology]]
-[[Category:Hematology]]
 {{WikiDoc Help Menu}}
 {{WikiDoc Sources}}
+ [[Category:Up-To-Date]]
+[[Category:Oncology]]
+[[Category:Medicine]]
+[[Category:Neurology]]
+[[Category:Neurosurgery]]