Label
severe chronic neutropenia
Synonyms
neutropenia, severe chronic
Synonym type
exact
Definition
Severe chronic neutropenia is a heterogeneous group of rare congenital and acquired disorders characterized by a persistent absolute neutrophil count (ANC) below 0.5 × 10⁹/L for at least 3 months, resulting in an increased risk of recurrent and potentially life-threatening bacterial and fungal infections. The major clinical subtypes include congenital, cyclic, idiopathic, and autoimmune neutropenia, which share common diagnostic evaluation, treatment strategies, and long-term follow-up. The Severe Chronic Neutropenia International Registry (SCNIR) has followed thousands of patients over decades.
Parent term
chronic neutropenia
Children term(s)
severe congenital neutropenia, cyclic neutropenia , chronic idiopathic neutropenia, chronic autoimmune neutropenia
ORCID Identifier
0000-001-9310-0163
Website URL
https://rarediseases.org/rare-diseases/severe-chronic-neutropenia/
Additional comments
We have a NORD report. Thank you, Gioconda Alyea
Label
severe chronic neutropenia
Synonyms
neutropenia, severe chronic
Synonym type
exact
Definition
Severe chronic neutropenia is a heterogeneous group of rare congenital and acquired disorders characterized by a persistent absolute neutrophil count (ANC) below 0.5 × 10⁹/L for at least 3 months, resulting in an increased risk of recurrent and potentially life-threatening bacterial and fungal infections. The major clinical subtypes include congenital, cyclic, idiopathic, and autoimmune neutropenia, which share common diagnostic evaluation, treatment strategies, and long-term follow-up. The Severe Chronic Neutropenia International Registry (SCNIR) has followed thousands of patients over decades.
Parent term
chronic neutropenia
Children term(s)
severe congenital neutropenia, cyclic neutropenia , chronic idiopathic neutropenia, chronic autoimmune neutropenia
ORCID Identifier
0000-001-9310-0163
Website URL
https://rarediseases.org/rare-diseases/severe-chronic-neutropenia/
Additional comments
We have a NORD report. Thank you, Gioconda Alyea