Myelofibrosis medical therapy: Difference between revisions
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==Overview== | ==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, | [[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|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== | ||
===Treatment for Anemia=== | ===Treatment for Anemia=== | ||
*'''Red blood cell transfusion''': The profound [[anemia]] that develops in this disease usually requires 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> | *'''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 (RBC) survival, which can be markedly decreased in some 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> | *'''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 (RBCs). | *'''Androgen therapy''': [[Danazol]] can be given to stimulate the production of [[Red blood cells|red blood cells (RBCs)]]. | ||
*'''Erythropoietic growth factors''': [[Erythropoietin]] and [[darbepoetin]] can help if patients are not dependent on transfusion. | *'''Erythropoietic growth factors''': [[Erythropoietin]] and [[darbepoetin]] can help if [[patients]] are not dependent on [[transfusion]]. | ||
*'''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.<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> | *'''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> | ||
===Janus kinase (JAK) Inhibitor Therapy=== | ===Janus kinase (JAK) Inhibitor Therapy=== | ||
*[[Ruxolitinib]], an inhibitor of '' | *[[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]] 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> | ||
===Treatment for Splenomegaly=== | ===Treatment for Splenomegaly=== | ||
*'''''JAK2'' inhibitor therapy''': [[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> | *'''''JAK2'' inhibitor therapy''': [[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> | ||
*'''Hydroxyurea''': Hydroxyurea can reduce the splenomegaly but may have a potential leukemogenic effect. | *'''Hydroxyurea''': [[Hydroxyurea]] can reduce the [[splenomegaly]] but may have a potential leukemogenic effect. | ||
*'''Chemotherapy''': Drugs like [[thalidomide]], [[lenalidomide]], or [[cladribine]] may also reduce the spleen size, and relieve the pain symptoms. | *'''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. | *'''Radiation therapy''': [[Radiation]] may be used to reduce the size of the [[spleen]], when [[splenectomy]] is contraindicated. | ||
*'''Splenectomy''': Splenectomy can also be performed. | *'''Splenectomy''': [[Splenectomy]] can also be performed. | ||
==References== | ==References== | ||
Revision as of 21:31, 11 December 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
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).
- Erythropoietic growth factors: Erythropoietin and darbepoetin can help if patients are not dependent on transfusion.
- 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]
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]
Treatment for Splenomegaly
- JAK2 inhibitor therapy: ruxolitinib can provide temporary relief.[1][7]
- Hydroxyurea: Hydroxyurea can reduce the splenomegaly but may have a potential leukemogenic effect.
- 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.
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.