The diagnosis and treatment of six patients with infantile pyknocytosis are reported. The clinical course and the diagnostic work-up are shown. All the patients necessitated therapy: phototherapy in five patients and one red blood cell transfusion in all of them; in two patients, because of the persistence of haemolysis and pyknocytosis with inadequate erythropoiesis, a brief course of erythropoietin treatment was undertaken with an excellent response and no side effects. Infantile pyknocytosis is a rare but not infrequent disease: physicians caring for a neonate with haemolytic anaemia should not forget this disease in the differential diagnosis.
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