Neutropenia

Neutropenia
Updated: 08/03/2020
© Jun Wang, MD, PhD

General features
  • Heterogeneous group of disorders of decrease in circulating neutrophils
  • Grading by absolute neutrophil count: Mild (1000-1500/mm3), Moderate (500-1000/ mm3), and severe (<500/ mm3)
  • Risk of serious infection, especially when absolute neutrophil count (ANC)  (< 500/ mm3)
  • Agranulocytosis: Absence of neutrophils in peripheral blood, usually applied if ANC < 100/ mm3
  • Granulocytopenia: Reduced number of blood granulocytes, namely neutrophils, eosinophils, and basophils
Clinical features
  • Risk of infection
Key pathogenesis
  • Reduced production: Toxicity of medication or other substances probably to stem cells, or immune response
  • Ineffective hematopoiesis: MDS, megaloblastic anemias, etc
  • Increased destruction or sequestration: Immune mediated neutrophilic injuries, splenomegaly
  • Increased peripheral utilization: Infection, etc
  •  Congenital: Isolated neutropenia, etc
Benign ethnic neutropenia
  • African descent
  • No increased risk for infection
  • Associated with polymorphism of Duffy antigen receptor
  • No treatment needed
Autoimmune neutropenia
  • Most common in children
  • Due to anti-neutrophil antibodies
  • Commonly seen targets by autoimmune IgG: Neutrophil glycosylated isoforms of FC gamma receptor IIIb (or CD 16b), human neutrophil antigen 1 (HNA1) and, less commonly, HNA4
  • Recurrent infections
  • Usually self limited in infancy, last 2-3 years 
  • Late childhood and adults AIN: more common in female, rare self remission
  • Differential diagnosis: Other primary immunodeficiency disorders (usually has lymphocytopenia and/or abnormal immunoglobulin levels)
Chediak-Higashi syndrome 


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