Rare diseases · Sign or symptom
Decreased total neutrophil count
Low blood neutrophil count
HP:0001875
What it means
Abnormal decrease of absolute number of neutrophils in the blood, per microlitre, compared to a reference range for a given sex and age-group.
Mild neutropenia is defined as an absolute neutrophil count of less than 1500 cells per microliter of blood, moderate less than 1000 cells per microliter of blood and, and severe is less than 500 cells per microliter of blood.
Rare diseases that can present with this67
Very common80–99%
14- 3-methylglutaconic aciduria-neonatal cataract-neurologic involvement-congenital neutropenia syndrome
- Alpha-thalassemia-myelodysplastic syndrome
- Cartilage-hair hypoplasia
- Cohen syndrome
- Felty syndrome
- Hermansky-Pudlak syndrome
- Pearson syndrome
- Primary immunodeficiency syndrome due to P14/LAMTOR2 deficiency
- Shwachman-Diamond syndrome
- SLC35A1-CDG
- Toxic epidermal necrolysis
- Transient predisposition to invasive pyogenic bacterial infection
- WHIM syndrome
- X-linked severe congenital neutropenia
Common30–79%
18- Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
- Aspergillosis
- Barth syndrome
- Congenital progressive bone marrow failure-B-cell immunodeficiency-skeletal dysplasia syndrome
- DIAPH1-related sensorineural hearing loss-thrombocytopenia syndrome
- Familial hemophagocytic lymphohistiocytosis
- Fusariosis
- Griscelli syndrome type 2
- Hemophagocytic syndrome associated with an infection
- Imerslund-Gräsbeck syndrome
- Macrophage activation syndrome
- Microcephalic primordial dwarfism, Toriello type
- Necrotizing enterocolitis
- Schimke immuno-osseous dysplasia
- Sepsis in premature infants
- Transcobalamin II deficiency
- Wolcott-Rallison syndrome
- X-linked agammaglobulinemia
Sometimes5–29%
27- Acute promyelocytic leukemia
- Aggressive systemic mastocytosis
- Autosomal non-syndromic agammaglobulinemia
- Chédiak-Higashi syndrome
- Congenital enterovirus infection
- Fumaric aciduria
- Idiopathic aplastic anemia
- Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
and 19 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Low neutrophil count · Neutropenia · Neutropoenia · Peripheral neutropenia
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.