Other disorders involving the immune mechanism, not elsewhere classified

Description

Mastocytosis: A clonal myeloproliferative neoplasm characterized by the proliferation and accumulation of neoplastic mast cells in one or multiple organs or organ systems. It is a heterogeneous group of neoplasms, ranging from cutaneous proliferations which may regress spontaneously, to aggressive neoplasms associated with organ failure and short survival.

Data source
FinnGen phenocode D3_IMMUNEMECHANISMOTHER
Hospital Discharge registry ICD-10: D89
Cause of Death registry ICD-10: D89
Include D3_POLYCLOHYPERGAMMA, D3_CRYOGLOBU, D3_HYPERGAMMAGLONAS, D3_IMMUNEMECHANISMNAS
Level in the ICD-hierarchy 3
First defined in version DF2
Ontology
DOID 350
GWAS catalog 0009001
MESH D008415

Key figures

Sex All Female Male
Number of individuals 207 76 131
Unadjusted prevalence (%) 0.16 0.1 0.23
Mean age at first event (years) 50.56 46.27 53.05
Median number of events / individual 5 3 6

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 61.35 53.95 65.65
Case fatality at 5-years (%) 6.28 1.32 9.16

Associations