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Kostmann syndrome (Infantile genetic agranulocytosis), a disease characterized by recurrent
infections leading to death in infancy, shows an agranulocytosis with variable monocytosis,
eosinophilia in the peripheral blood, accompanied by the maturation arrest of the myelocytic
series at the promyelocyte-myelocyte level.
A 2 years 3 month old female patient with Kostmann syndrome is reported, who has
suffered from recurrent infections since 1 month of age. During... |