Primary Myelofibrosis

GPTKB entity

Statements (51)
Predicate Object
gptkbp:instanceOf gptkb:disease
myeloproliferative neoplasm
gptkbp:affects bone marrow
gptkbp:characterizedBy gptkb:anemia
splenomegaly
extramedullary hematopoiesis
bone marrow fibrosis
leukoerythroblastosis
gptkbp:complication gptkb:acute_myeloid_leukemia
infections
portal hypertension
gptkbp:diagnosedBy genetic testing
bone marrow biopsy
blood smear
gptkbp:firstDescribed 1951
gptkbp:hasEpidemiology 1-2 per 100,000 per year
gptkbp:hasOrphanetID ORPHA:732
gptkbp:hasPathologyFinding osteosclerosis
bone marrow fibrosis
megakaryocyte proliferation
reticulin fibrosis
teardrop-shaped red blood cells
https://www.w3.org/2000/01/rdf-schema#label Primary Myelofibrosis
gptkbp:ICD-10_code D47.4
gptkbp:inheritance sporadic
gptkbp:medianAge 65 years
gptkbp:MeSH_ID D009196
gptkbp:mortalityRate high
gptkbp:mutationAssociatedWith gptkb:JAK2_gene
CALR gene
MPL gene
gptkbp:OMIM 254450
gptkbp:onset adulthood
gptkbp:prevalence rare
gptkbp:prognosis variable
gptkbp:riskFactor male sex
older age
exposure to toxins
gptkbp:symptom fatigue
weight loss
night sweats
abdominal discomfort
gptkbp:synonym agnogenic myeloid metaplasia
chronic idiopathic myelofibrosis
idiopathic myelofibrosis
gptkbp:treatment gptkb:fedratinib
gptkb:ruxolitinib
gptkb:hydroxyurea
gptkb:allogeneic_stem_cell_transplantation
gptkbp:bfsParent gptkb:PMF
gptkbp:bfsLayer 6