Myeloproliferative neoplasm medical therapy: Difference between revisions

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Revision as of 18:17, 10 June 2018

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

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.^[1]

Medical Therapy

Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm.

Polycythemia vera


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

References

↑ National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19

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[cancergov-1] National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19

[1]

@@ Line 37: / Line 37: @@
 |-
 | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Primary myelofibrosis
+Ruxolitinib
 | style="padding: 5px 5px; background: #F5F5F5;" |
-Megakaryocyte
+Inhibits JAK2 (tyrosine kinase inhibitor)
 | style="padding: 5px 5px; background: #F5F5F5;" |
-''Major criteria'':
+mg PO twice daily
-*Presence of megakaryocyte proliferation and atypia with reticulin fibrosis
-*Not meeting criteria for other myeloproliferative neoplasms
-*Presence of ''JAK2'', ''CALR'', or ''MPL'' mutation
-''Minor criteria'':
-*Anemia
-*White blood cell count >11,000 per microliter.
-*Palpable splenomegaly
-*Elevated LDH
-*Leukoerythroblastic smear
-Diagnosis requires meeting all major criteria and at least 1 minor criterion.
-|-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Chronic myeloid leukemia
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Common myeloid progenitor
 | style="padding: 5px 5px; background: #F5F5F5;" |
-*Presence of BCR-ABL translocation (chromosomes 9 and 22)
+Weight gain, zoster, non-melanoma skin cancers, cytopenias
 |-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Chronic neutrophilic leukemia
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Neutrophil
-| style="padding: 5px 5px; background: #F5F5F5;" |
-*Peripheral blood white blood cell count > 25,000 per microliter with rare myeloblasts and no dysgranulopoiesis
-*Bone marrow hypercellularity with increased granulocytes and normal maturation and <5% myeloblasts
-*Not meeting criteria for other myeloproliferative neoplasms
-*Absence of genetic rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', or ''PCM1-JAK2''
-*Presence of ''CSF3R'' ''T618I'' or other characteristic mutation
-|-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Chronic eosinophilic leukemia
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Eosinophil
-| style="padding: 5px 5px; background: #F5F5F5;" |
-No formal W.H.O. criteria
-*Typically associated with >1,500 eosinophils per microliter in peripheral blood
-*Typically associated with rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', ''JAK2''
-|-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Myeloproliferative neoplasm, unclassifiable
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Variable
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Not meeting criteria for other subcategories
-|-
-| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
-Mastocytosis
-| style="padding: 5px 5px; background: #F5F5F5;" |
-Mast cell
-| style="padding: 5px 5px; background: #F5F5F5;" |
-''Major criteria'':
-*Dense multifocal aggregates of >15 mast cells in bone marrow or other organ
-''Minor criteria'':
-*Presence of ''c-kit'' ''D816V''mutation
-*Expression of CD2, CD25, or both on mast cells
-*Serum tryptase level >20ng/ml when patient is at baseline health
-*Atypical morphology or spindles in >25% of mast cells in bone marrow or other organ
-Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria.
 |}