Background
- Low concentration of circulating neutrophils
ANC
|
Classification
|
≥1500
|
Normal
|
1000-1499
|
Mild
|
500-999
|
Moderate
|
<500
|
Severe
|
0
|
Agranulocytosis
|
Etiologies
Congenital
- Constitutional neutropenia: mild (ANC>1000), chronic
- Benign ethnic neutropenia: mild (ANC>1000), chronic, usually in patients with Mediterranean or African ancestry
- Benign familial neutropenia: same as above, but hereditary and not associated with a specific ethnicity
- Cyclic neutropenia: hereditary, with severe neutropenia on a 21-day cycle since childhood
- Others: severe congenital neutropenia, Schwachman-Diamond syndrome, Fanconi anemia, dyskeratosis congenita, Chediak-Higash syndrome, myelokathesis, Griscelli syndrome II, and cartilage-hair hypoplasia
Acquired
- Infectious
- Active infection, particularly viral, but including severe sepsis from any cause; more common in the elderly
- Post-infectious neutropenia, most commonly in children after viral infections (particularly varicella, measles, rubella, influenza, hepatitis, EBV, or HIV)
- EBV and HIV can sometimes cause prolonged neutropenia; the rest are self-limited
- Bacterial infections, including Brucella, Rickettsiae, and mycobacteria
- Drug-induced neutropenia, including agranulocytosis or more mild neutropenia
- Autoimmune
- Primary: typically in patients less than 1 year old, can be severe, but usually resolves within 2 years
- Needs prophylactic antibiotics and GCSF
- Secondary: usually mild, in the context of a systemic autoimmune disease (with lupus being common and tends to reflect disease activity)
- Felty syndrome (triad of rheumatoid arthritis, splenomegaly, and neutropenia)
- Malignancy
- Dietary
- Chronic idiopathic neutropenia
Further Reading