Myeloproliferative neoplasm medical therapy: Difference between revisions

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Latest revision as of 04:07, 15 July 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2] Shyam Patel [3]

Overview

Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm. The mainstay of therapy for myeloproliferative neoplasm is chemotherapy, cytoreduction, aspirin, and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.

Medical Therapy

Treatment varies for each of the eight subtypes of myeloproliferative neoplasm. The treatment recommendations are based on guidelines by the National Comprehensive Cancer Network.

Polycythemia vera^[1]


Therapy	Mechanism of Action	Dosing	Adverse Effects

Aspirin	Irreversibly inhibits cyclooxygenase-1 and -2 (COX-1 and COX-2)	81mg PO daily	Mucosal bleeding Gastrointestinal bleeding
Hydroxyurea	Inhibits ribonucleotide reductase	20mg/kg PO daily	Anemia, thrombocytopenia, ulcerations, secondary cancers
Ruxolitinib	Inhibits JAK2 (tyrosine kinase inhibitor)	10mg PO twice daily	Weight gain, zoster, non-melanoma skin cancers, cytopenias
Phlebotomy	Mechanically removes red blood cells from circulation	Induction: 450cc blood removal daily until hematocrit < 45% Maintenance: One session every 2 months, with goal hematocrit < 45%	Iron deficiency anemia, fatigue, vasovagal episodes, pain at phlebotomy site

Essential thrombocythemia^[1]

Treatment of essential thrombocythemia is based on risk assessment and prognostication:


Prognostic Group	Defining Features	Therapy

Very low risk^[2]	No history of thrombosis Age < 60 JAK2 or MPL wild-type	Observation or Aspirin once daily
Low risk^[2]	No history of thrombosis Age < 60 JAK2 or MPL mutation present	Aspirin once daily or Aspirin twice daily
Intermediate risk^[2]	No history of thrombosis Age > 60 JAK2 or MPL wild-type	Aspirin once daily plus hydroxyurea
High risk^[2]	History of thrombosis, or Age > 60 with JAK2 or MPL mutation present	Aspirin twice daily plus hydroxyurea or Hydroxyurea, plus systemic anticoagulation


Therapy	Mechanism of Action	Dosing	Adverse Effects

Anagrelide	Inhibits phosphodiesterase 3 (PDE-3) Inhibits release of arachidonic acid from phospholipase A2 Disrupts maturation of megakaryocytes	0.5mg PO every 6 hours or 1mg every 12 hours	Headache, palpitations, diarrhea, edema, nausea
Hydroxyurea	Inhibits ribonucleotide reductase	20mg/kg PO daily	Anemia, thrombocytopenia, ulcerations, secondary cancers
Aspirin	Irreversibly inhibits cyclooxygenase-1 and -2 (COX-1 and COX-2)	81mg PO twice daily	Mucosal bleeding Gastrointestinal bleeding
Ruxolitinib	Inhibits JAK2 (tyrosine kinase inhibitor)	10mg PO twice daily	Weight gain, zoster, non-melanoma skin cancers, cytopenias
Plateletpheresis	Mechanically removes platelets from circulation	Daily until platelet count returns to normal range	Hypotension, thrombocytopenia
Pegylated interferon alpha 2a	Immunomodulatory agent; anti-angiogenic agent	45mcg/week	Hypotension, infusion reaction

Primary myelofibrosis^[1]


Therapy	Mechanism of Action	Dosing	Adverse Effects

Hydroxyurea	Inhibits ribonucleotide reductase	20mg/kg PO daily	Anemia, thrombocytopenia, ulcerations, secondary cancers
Ruxolitinib	Inhibits JAK2 (tyrosine kinase inhibitor)	10mg PO twice daily	Weight gain, zoster, non-melanoma skin cancers, cytopenias
Radiation therapy	Unknown mechanism	0.5 Gy to spleen 5 days weekly	Fatigue, secondary cancers, nausea, cytopenias

Chronic myeloid leukemia^[3]


Therapy	Mechanism of Action	Dosing	Adverse Effects

Imatinib	Inhibits BCR-ABL tyrosine kinase	400mg PO daily	Edema, periorbital swelling, nausea, anasarca
Dasatinib	Inhibits BCR-ABL tyrosine kinase	100mg PO daily	Pleural effusions, edema, periorbital swelling, facial edema
Nilotinib	Inhibits BCR-ABL tyrosine kinase	400mg PO twice daily	Occlusive arterial disease, peripheral vascular disease, cytopenias, nausea
Bosutinib	Inhibits BCR-ABL tyrosine kinase	400mg PO daily	Edema, chest pain, fatigue, diarrhea
Ponatinib	Inhibits BCR-ABL tyrosine kinase; effective against the T315I kinase domain mutation in BCR-ABL	45mg PO daily	Hypertension, arterial ischemia, fatigue, constipation
Omacetaxine	Inhibits protein synthesis	Induction: 1.25mg/m2 subQ twice daily for 14 days of a 28-day cycle Maintenance: 1.25mg/m2 subQ twice daily for 7 days of a 28-day cycle	Peripheral edema, fatigue, nausea, thrombocytopenia

Chronic neutrophilic leukemia^[1]


Therapy	Mechanism of Action	Dosing	Adverse Effects

Hydroxyurea	Inhibits ribonucleotide reductase	20mg/kg PO daily	Anemia, thrombocytopenia, ulcerations, secondary cancers
Pegylated interferon alpha 2a	Immunomodulatory agent; anti-angiogenic agent	45mcg/week	Hypotension, infusion reaction

Chronic eosinophilic leukemia^[1]


Therapy	Mechanism of Action	Dosing	Adverse Effects

Imatinib^[4]	Tyrosine kinase inhibitor that inhibits wild-type c-kit; not effective for c-kit D816V mutation	400mg PO daily	Edema, periorbital swelling, nausea, anasarca
Corticosteroids	Immunosuppressive agent; inhibits IL-2 and COX2	Variable; typical dose is 1mg/kg/day	Infections, cataract, glaucoma, bone loss, muscular atrophy

Systemic mastocytosis


Therapy	Mechanism of Action	Dosing	Adverse Effects

Imatinib^[4]	Tyrosine kinase inhibitor that inhibits wild-type c-kit; not effective for c-kit D816V mutation	400mg PO daily	Edema, periorbital swelling, nausea, anasarca
Midostaurin^[4]	Tyrosine kinase inhibitor that inhibits wild-type c-kit and mutant c-kit D816V	10mg PO twice daily	Nausea, hypocalcemia, mucositis, headache, epistaxis, hypernatremia

References

↑ ^1.0 ^1.1 ^1.2 ^1.3 ^1.4 Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT; et al. (2017). "NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018". J Natl Compr Canc Netw. 15 (10): 1193–1207. doi:10.6004/jnccn.2017.0157. PMID PMID28982745 Check |pmid= value (help).
↑ ^2.0 ^2.1 ^2.2 ^2.3 Tefferi A, Vannucchi AM, Barbui T (2018). "Essential thrombocythemia treatment algorithm 2018". Blood Cancer J. 8 (1): 2. doi:10.1038/s41408-017-0041-8. PMC 5802626. PMID 29321520.
↑ Pallera A, Altman JK, Berman E, Abboud CN, Bhatnagar B, Curtin P; et al. (2016). "NCCN Guidelines Insights: Chronic Myeloid Leukemia, Version 1.2017". J Natl Compr Canc Netw. 14 (12): 1505–1512. PMID PMID27956535 Check |pmid= value (help).
↑ ^4.0 ^4.1 ^4.2 Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O; et al. (2017). "Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future". Cancer Res. 77 (6): 1261–1270. doi:10.1158/0008-5472.CAN-16-2234. PMC 5354959. PMID 28254862.

Template:WH Template:WS

[pmidPMID28982745-1] 1.0 ^1.1 ^1.2 ^1.3 ^1.4 Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT; et al. (2017). "NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018". J Natl Compr Canc Netw. 15 (10): 1193–1207. doi:10.6004/jnccn.2017.0157. PMID PMID28982745 Check |pmid= value (help).

[pmid29321520-2] 2.0 ^2.1 ^2.2 ^2.3 Tefferi A, Vannucchi AM, Barbui T (2018). "Essential thrombocythemia treatment algorithm 2018". Blood Cancer J. 8 (1): 2. doi:10.1038/s41408-017-0041-8. PMC 5802626. PMID 29321520.

[pmidPMID27956535-3] Pallera A, Altman JK, Berman E, Abboud CN, Bhatnagar B, Curtin P; et al. (2016). "NCCN Guidelines Insights: Chronic Myeloid Leukemia, Version 1.2017". J Natl Compr Canc Netw. 14 (12): 1505–1512. PMID PMID27956535 Check |pmid= value (help).

[pmid28254862-4] 4.0 ^4.1 ^4.2 Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O; et al. (2017). "Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future". Cancer Res. 77 (6): 1261–1270. doi:10.1158/0008-5472.CAN-16-2234. PMC 5354959. PMID 28254862.

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@@ Line 3: / Line 3: @@
 {{CMG}} {{AE}} {{MJK}} {{shyam}}
 ==Overview==
-The mainstay of therapy for myeloproliferative neoplasm is [[chemotherapy]], [[aspirin]], and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19</ref>
+Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm. The mainstay of therapy for myeloproliferative neoplasm is [[chemotherapy]], [[cytoreduction]], [[aspirin]], and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.
 ==Medical Therapy==
-Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm.
+Treatment varies for each of the eight subtypes of myeloproliferative neoplasm. The treatment recommendations are based on guidelines by the National Comprehensive Cancer Network.
-===Polycythemia vera===
+===[[Polycythemia vera]]<ref name="pmidPMID28982745">{{cite journal| author=Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT et al.| title=NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018. | journal=J Natl Compr Canc Netw | year= 2017 | volume= 15 | issue= 10 | pages= 1193-1207 | pmid=PMID28982745 | doi=10.6004/jnccn.2017.0157 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28982745  }} </ref>===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
@@ Line 56: / Line 57: @@
 |}
-===Essential thrombocythemia===
+===[[Essential thrombocythemia]]<ref name="pmidPMID28982745">{{cite journal| author=Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT et al.| title=NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018. | journal=J Natl Compr Canc Netw | year= 2017 | volume= 15 | issue= 10 | pages= 1193-1207 | pmid=PMID28982745 | doi=10.6004/jnccn.2017.0157 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28982745  }} </ref>===
 Treatment of essential thrombocythemia is based on risk assessment and prognostication:
@@ Line 177: / Line 178: @@
 |}
-===Primary myelofibrosis===
+===[[Primary myelofibrosis]]<ref name="pmidPMID28982745">{{cite journal| author=Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT et al.| title=NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018. | journal=J Natl Compr Canc Netw | year= 2017 | volume= 15 | issue= 10 | pages= 1193-1207 | pmid=PMID28982745 | doi=10.6004/jnccn.2017.0157 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28982745  }} </ref>===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
 |valign=top|
@@ Line 214: / Line 215: @@
 |}
-===Chronic myeloid leukemia===
+===[[Chronic myeloid leukemia]]<ref name="pmidPMID27956535">{{cite journal| author=Pallera A, Altman JK, Berman E, Abboud CN, Bhatnagar B, Curtin P et al.| title=NCCN Guidelines Insights: Chronic Myeloid Leukemia, Version 1.2017. | journal=J Natl Compr Canc Netw | year= 2016 | volume= 14 | issue= 12 | pages= 1505-1512 | pmid=PMID27956535 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27956535  }} </ref>===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
@@ Line 263: / Line 264: @@
 Ponatinib
 | style="padding: 5px 5px; background: #F5F5F5;" |
-Inhibits BCR-ABL tyrosine kinase
+Inhibits BCR-ABL tyrosine kinase; effective against the ''T315I'' kinase domain mutation in BCR-ABL
 | style="padding: 5px 5px; background: #F5F5F5;" |
 mg PO daily
@@ Line 281: / Line 282: @@
 |}
-===Chronic neutrophilic leukemia===
+===[[Chronic neutrophilic leukemia]]<ref name="pmidPMID28982745">{{cite journal| author=Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT et al.| title=NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018. | journal=J Natl Compr Canc Netw | year= 2017 | volume= 15 | issue= 10 | pages= 1193-1207 | pmid=PMID28982745 | doi=10.6004/jnccn.2017.0157 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28982745  }} </ref>===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
 |valign=top|
@@ Line 310: / Line 311: @@
 |}
-===Chronic eosinophilic leukemia===
+===[[Chronic eosinophilic leukemia]]<ref name="pmidPMID28982745">{{cite journal| author=Mesa RA, Jamieson C, Bhatia R, Deininger MW, Fletcher CD, Gerds AT et al.| title=NCCN Guidelines Insights: Myeloproliferative Neoplasms, Version 2.2018. | journal=J Natl Compr Canc Netw | year= 2017 | volume= 15 | issue= 10 | pages= 1193-1207 | pmid=PMID28982745 | doi=10.6004/jnccn.2017.0157 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28982745  }} </ref>===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
 |valign=top|
@@ Line 328: / Line 329: @@
 Edema, periorbital swelling, nausea, anasarca
 |-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold"
+| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
 Corticosteroids
 | style="padding: 5px 5px; background: #F5F5F5;" |
@@ Line 339: / Line 340: @@
 |}
-===Systemic mastocytosis===
+===[[Systemic mastocytosis]]===
 {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
 |valign=top|

Myeloproliferative neoplasm medical therapy: Difference between revisions

Latest revision as of 04:07, 15 July 2018

Overview

Medical Therapy

Polycythemia vera[1]

Essential thrombocythemia[1]

Primary myelofibrosis[1]

Chronic myeloid leukemia[3]

Chronic neutrophilic leukemia[1]

Chronic eosinophilic leukemia[1]