Kostmann syndrome

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Kostmann syndrome also known as Severe Congenital Neutropenia (SCN) is a rare inherited form of Severe Chronic Neutropenia usually detected soon after birth. It was discovered in 1956 by Swedish doctor Kostmann. A absolute neutrophil count (ANC) chronically less than 500/mm3 is the main sign of Kostmann's. A standard bone marrow test can give correct diagnosis.

[edit] References

  • KOSTMANN R (1956). "Infantile genetic agranulocytosis; agranulocytosis infantilis hereditaria.". Acta Paediatr 45 (Suppl 105): 1-78. PMID 13326376.

[edit] See also

[edit] External Links

Kostmann defined at:

eMedicine
Medicine Net
Neutropenia.ca

Support Groups:

Severe Chronic Neutropenia International Registry
Neutropenia.ca
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