Unifying Concepts

Classification of Neutropenia

Acquired neutropenia

    • Postinfectious: varicella, measles, rubella, hepatitis A and B, mononucleosis, influenza, cytomegalovirus, parvovirus, acquired immunodeficiency syndrome (AIDS), S. aureus, brucellosis, tularemia, rickettsia, Mycobacterium tuberculosis, sepsis.
    • Drug induced: Antineoplastic agents, procainamide, antithyroid drugs, sulphasalazine, phenothiazines, semisynthetic penicillins, nonsteroidal anti-inflammatory agents, aminopyrine derivatives, benzodiazepines, barbiturates, gold compounds, sulfonamides, propranolol, dipyridamole, digoxin, acetyldigoxin, sulfamethoxizole, anticonvulsants
    • Benign familial neutropenia
    • Chronic benign neutropenia of childhood
    • Chronic idiopathic neutropenia
    • Autoimmune neutropenia
    • Isoimmune neutropenia
    • Neutropenia associated with immunologic abnormalities
    • Neutropenia associated with metabolic diseases
    • Neutropenia due to increased margination
    • Nutritional deficiency

Intrinsic defects

  • Kostmann syndrome (severe infantile agranulocytosis)
  • Myelokathexis/neutropenia with tetraploid nuclei
  • Cyclic neutropenia
  • Shwachman-Diamond-Oski syndrome
  • Chediak-Higashi syndrome
  • Reticular dysgenesis
  • Dyskeratosis congenital


Clinically Significant Neutrophil Counts


Clinical Significance




No significant propensity to infection. Fevers can be managed on an outpatient basis.


Some propensity to infection. Occasionally, fever can be managed on an outpatient basis.


Significant propensity to infection. Always should be managed as inpatient with parenteral antibiotics. Few clinical signs of infection.

ANC; Absolute Neutrophil Counts

These rules apply strictly for neutropenia with hypoplastic marrow, early myeloid arrest, and decreased granulocyte reserve pools. There is more latitude for clinical judgment in neutropenias with normocellular marrow. The only regular exception to these rules is documented chronic benign neutropenia of childhood.



  1. Kyono W, Coates TD. A practical approach to neutrophil disorders. Pediatr Clin North Am. 2002 Oct;49(5):929-71, viii. [Medline]


Created: Oct 23, 2005

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