Definition
MDS/MPN with neutrophilia is an overlap disorder (e.g. like CMML)
Presents with a dysplastic neutrophil leukocytosis, often with additional multi-lineage dysplasia
BCR-ABL1, PDGFRA and PDGFRB mutations are not present
Epidemiology
Rare disorder, typically of older age
Median age at diagnosis: 60-70
Presentation
Symptomatic cytopenias
Leukocytosis
Hepatosplenomegaly
Diagnostics
Blood
WBC >13 - mainly neutrophils but with myelocytes, promyelocytes and monocytes present
Marked dysplastic change
Basophilia is not prominent
Blasts <5%
Marrow morphology
Hypercellular
Marked dysgranulopoeisis +/- other lineage dysplasias (common)
Blasts increased but <20%
Flow Cytometry
No specific immunophenotype
Cytogenetics/Molecular
Abnormal karyotype present in most patients, e.g. +8, del(20q)
SETBP1, NRAS and KRAS mutations may be present
Management
Fit patients: Cytoreduction followed by allogeneic stem cell transplant
Non-transplant eligible: Cytoreduction, Azactidine
Prognosis: Median survival 1-2 years