Differential diagnoses

Isolated neutropenia may be the presenting feature of myelodysplasia, aplastic anaemia, Fanconi's anaemia or acute leukaemia but these conditions will usually be associated with other haematological abnormalities.

Post-infectious (most usually post-viral) neutropenia may last several weeks and may be followed by prolonged immune neutropenia.

Severe sepsis particularly at the extremes of life.

Drugs—cytotoxic agents, and many others, notably phenothiazines, many antibiotics, NSAIDs, anti-thyroid agents and psychotropic agents. Recovery of neutrophils usually starts within a few days of stopping the offending drug.

Autoimmune neutropenia due to anti-neutrophil antibodies may occur in isolation or in association with haemolytic anaemia, immune thrombocytopenia or SLE.

Felty's syndrome neutropenia is accompanied by seropositive rheumatoid arthritis, and splenomegaly.

Chronic benign neutropenia of infancy and childhood is associated with fever and infection but resolves by age 4, probably also has an immune basis.

Benign familial neutropenia is a feature of rare families and of certain racial groups, notably negroes, is associated with mild neutropenia but no propensity to infection.

Chronic idiopathic neutropenia is a diagnosis of exclusion, associated with severe neutropenia but often a benign course.

Cyclical neutropenia is a condition of childhood onset and dominant inheritance characterised by severe neutropenia, fever, stomatitis and other infections occurring with a periodicity of ~4 weeks.

Hereditary causes (less common) include Kostmann syndrome (ffl p459), Shwachman-Diamond-Oski syndrome (ffl p459), Chediak-Higashi syndrome (ffl p465), reticular dysgenesis and dyskeratosis congenita.

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