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{{Myelofibrosis}}
{{Myelofibrosis}}
{{CMG}}{{AE}}{{SR}}
{{CMG}}{{AE}}{{Sab}}{{SR}}


==Overview==
==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==
Asymptomatic low-risk patients should be followed with a watchful waiting approach. Some individuals remain symptom-free for years. The development of [[anemia|symptomatic anemia]], [[leukocytosis|marked leukocytosis]], [[night sweats|drenching night sweats]], [[weight loss]], [[fever]], or [[splenomegaly|symptomatic splenomegaly]] would warrant therapeutic intervention.<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>
===Treatment for Anemia===
===Treatment for Anemia===
*'''Red blood cell transfusion''':The profound [[anemia]] that develops in this disease usually requires red blood cell transfusion. Red blood cell survival is markedly decreased in some patients; this can sometimes be treated with glucocorticoids.<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>
'''Red blood cell transfusion'''
*'''Androgen therapy''': [[Danazol]] may stimulate the production of red blood cells and decrease the anemia.
*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>
*'''Erythropoietic growth factors''': [[Erythropoietin]] and [[darbepoetin]] are less likely to help when patients are transfusion dependent or manifest a serum erythropoietin level greater than 125 U/L.
'''Glucocorticoids'''
*'''Thalidomide, lenalidomide, or pomalidomide''': These drugs may help in elevating the red blood cell counts, thus improving anemia, and reverse the splenomegaly. However, patients on thalidomide, lenalidomide, or pomalidomide require prophylaxis for avoiding thrombosis and careful monitoring for 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>
*[[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


===''JAK2'' Inhibitor Therapy===
===Janus kinase (JAK) Inhibitor Therapy===
*[[Ruxolitinib]], an inhibitor of ''[[JAK1]]'' and ''[[JAK2]]'', can reduce the [[splenomegaly]] and the debilitating symptoms of [[weight loss]], [[fatigue]], and [[night sweats]] for patients with ''JAK2''-positive or ''JAK2''-negative primary myelofibrosis, post–essential thrombocythemia myelofibrosis, or post–polycythemia vera myelofibrosis.
*[[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.


===Treatment for Splenomegaly===
===Treatment for Splenomegaly===
*'''''JAK2'' inhibitor therapy''': Painful splenomegaly can be treated temporarily with [[ruxolitinib]].
'''''JAK2'' inhibitor therapy'''
*'''Hydroxyurea''': Hydroxyurea can reduce the splenomegaly.
*[[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.
*'''Chemotherapy''': Drugs like [[thalidomide]], [[lenalidomide]], or [[cladribine]] may reduce the spleen size, and relieve the pain symptoms associated with it.
'''Hydroxyurea'''
*'''Radiation therapy''': [[Radiation]] may be used to kill the cells and reduce the size of the spleen, when [[splenectomy]] is contraindicated.
*[[Hydroxyurea]] can reduce the [[splenomegaly]] but may have a potential leukemogenic effect.
*'''Splenectomy''': It's a surgical procedure.
*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==
==References==
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Latest revision as of 22:25, 31 January 2019

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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

Glucocorticoids

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

Thalidomide, lenalidomide, or pomalidomide

Janus kinase (JAK) Inhibitor Therapy

  • 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

Radiation therapy

Splenectomy

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. ↑ 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.
  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.


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