Myeloproliferative neoplasm medical therapy

Myeloproliferative Neoplasm Microchapters
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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.

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
Primary myelofibrosis	Megakaryocyte	Major criteria: 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.
Chronic myeloid leukemia	Common myeloid progenitor	Presence of BCR-ABL translocation (chromosomes 9 and 22)
Chronic neutrophilic leukemia	Neutrophil	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
Chronic eosinophilic leukemia	Eosinophil	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
Myeloproliferative neoplasm, unclassifiable	Variable	Not meeting criteria for other subcategories
Mastocytosis	Mast cell	Major criteria: Dense multifocal aggregates of >15 mast cells in bone marrow or other organ Minor criteria: Presence of c-kit D816Vmutation 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.