Myeloproliferative neoplasm medical therapy

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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. 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. 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.
  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).
  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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