Metadata

Name Agranulocytosis (agranulocytosis)
Category Haematological or immunological
Sex
Adult Yes
UUID 68bfae22-ef38-4abc-bfe9-91b44d596855
Definition Agranulocytosis is a condition in which the absolute neutrophil count (ANC) is less than 100 neutrophils per microlitre of blood. People with this condition are at a very high risk of severe infection.

Ontologies

Disease Ontology (DOID) DOID_12987
GWAS Catalogue HP_0012235
MeSH D000380
SNOMED-CT 17182001
FinnGen D3_AGRANULOCYTOSIS

Key Summary Statistics

Cases from any source

Total 8320
Female (%) 4972 (60%)
Male (%) 3348 (40%)

Year first recorded

agranulocytosis year of first event

Cases linked in all UK Biobank sources

Cross-source concordance

Vertical bars correspond to number of cases per source/intersection; horizontal bars to left correspond to total number of cases per source.

Cross-sourced agranulocytosis cases

Incidence & prevalence

Sex and age-stratified incidence and prevalence of agranulocytosis

Baseline prevalence stratified by sex

Female 77.68 (71.77,83.59)
Male 38.78 (34.22,43.34)

Incidence stratified by sex

Female 13.29 (12.33,14.24)
Male 10.18 (9.27,11.09)

Incidence stratified by age

Ages 40-44 6.57 (4.97,8.17)
Ages 45-49 7.99 (6.48,9.51)
Ages 50-54 11.17 (9.51,12.82)
Ages 55-59 13.48 (11.83,15.12)
Ages 60-64 13.91 (12.47,15.35)
Ages 65-69 13.48 (11.86,15.1)

Period prevalence

Methods

Comparison with general population

Sex-standardised prevalence from CALIBER study (Kuan et al. 2019)

CALIBER and UKB agranulocytosis prevalence comparison for multiple age groups

Population Ages 40-49 Ages 50-59 Ages 60-69
UK Biobank 84 (75,93) 121 (113,130) 149 (141,157)
CALIBER 51 (49,53) 85 (82,87) 120 (116,123)

Stratified by age and Townsend deprivation status

Deprivation status Ages 40-49 Ages 50-59 Ages 60-69
Least deprived 80(61,104) 122(104,142) 156(140,175)
Low 72(54,94) 117(99,137) 142(126,160)
Medium 77(59,99) 110(93,130) 149(132,168)
High 106(86,129) 115(98,134) 148(130,167)
Most deprived 80(64,99) 143(124,164) 151(132,171)

Stratified by age and country

Country Ages 40-49 Ages 50-59 Ages 60-69
England 81(71,91) 124(115,134) 148(139,157)
Scotland 97(72,128) 98(79,120) 143(121,166)
Wales 96(65,137) 134(105,168) 169(140,203)

Risk factors analysis

‘Healthy’ BMI, smoking status ‘never’, or no hypertension (absence of hypertension records) were considered as reference values.

Hazard Ratio (HR) values with P < 0.0002 were considered to show significant association with disease onset after correction for multiple-testing using the Bonferroni method

N=153321, Events=259

Factor Level Hazard Ratio P value
BMI overweight 1.47 (1.07, 2.01) 0.0165
BMI obese 2.46 (1.81, 3.35) 1.19e-08
BMI underweight 0 (0, Inf) 0.998
smoking previous 1.06 (0.81, 1.39) 0.676
smoking current 0.75 (0.46, 1.22) 0.25
hypertension yes 0.83 (0.58, 1.19) 0.31

Risk factors analysis forest plot

Definition

UK Biobank field id Description Code Value
20002 Non-cancer illness code, self-reported 1448 neutropenia/lymphopenia
41202 Diagnoses - main ICD10 D70 Agranulocytosis
41204 Diagnoses - secondary ICD10 D70 Agranulocytosis
40001 Underlying (primary) cause of death: ICD10 D70 Agranulocytosis
40002 Contributory (secondary) causes of death: ICD10 D70 Agranulocytosis
42040 GP clinical event records 42H2.00 Leucopenia - low white count
42040 GP clinical event records 42H2.11 Leucopenia
42040 GP clinical event records 42H4.00 Agranulocytosis
42040 GP clinical event records 42J2.00 Neutropenia
42040 GP clinical event records D400000 Idiopathic agranulocytosis
42040 GP clinical event records D400011 Idiopathic neutropenia
42040 GP clinical event records D400100 Primary splenic neutropenia
42040 GP clinical event records D400200 Agranulocytosis - drug induced
42040 GP clinical event records D400211 Neutropenia - drug induced
42040 GP clinical event records D400312 Neutropenia due to irradiation
42040 GP clinical event records D400400 Agranulocytosis due to infection
42040 GP clinical event records D400411 Neutropenia due to infection
42040 GP clinical event records D400600 Drug-induced neutropenia
42040 GP clinical event records D400800 Acquired neutropenia NEC
42040 GP clinical event records D400811 Acquired agranulocytosis NEC
42040 GP clinical event records D400900 Cyclical neutropenia
42040 GP clinical event records D400A00 Leucopenia
42040 GP clinical event records D400.00 Agranulocytosis
42040 GP clinical event records D400.11 Kostmann’s syndrome
42040 GP clinical event records D400.12 Neutropenia
42040 GP clinical event records D400y00 Other specified agranulocytosis
42040 GP clinical event records D400z00 Agranulocytosis NOS
42040 GP clinical event records 42H4. Agranulocytosis
42040 GP clinical event records 42H2. Leucopenia
42040 GP clinical event records XE14G Acquired neutropenia NEC