Essential (haemorrhagic) thrombocythaemia

Description

primary myelofibrosis: Myelofibrosis with myeloid metaplasia is a myeloproliferative disease with annual incidence of approximately 1 case per 100,000 individuals and age at diagnosis around 60 (an increased prevalence is noted in Ashkenazi Jews). Clinical manifestations depend on the type of blood cell affected and may include anemia, pallor, splenomegaly, hypermetabolic state, petechiae, ecchymosis, bleeding, lymphadenopathy, hepatomegaly, portal hypertension.

Data source
FinnGen phenocode THROMBOCYTAEMIA
Hospital Discharge registry ICD-10: D473, ICD-9: 2387B, ICD-8: 2872
Cause of Death registry ICD-10: D473, ICD-9: 2387B, ICD-8: 2872
First defined in version DF2, additional
Ontology
DOID 4971
GWAS catalog 0002430
MESH D013920

Key figures

Sex All Female Male
Number of individuals 280 167 113
Unadjusted prevalence (%) 0.16 0.17 0.15
Mean age at first event (years) 59.52 59.04 60.21
Case fatality at 5-years (%) 5.0 2.99 7.96

Longitudinal metrics

Sex All Female Male
Median nb. of events per indiv. 8 6 10
Recurrence at 6 months (%) 73.21 71.86 75.22

Associations

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