|
gptkbp:instanceOf
|
gptkb:disease
gptkb: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
|
|
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
|
8
|
|
https://www.w3.org/2000/01/rdf-schema#label
|
Primary Myelofibrosis
|