European Society for Paediatric Research Abstract


Abstract 22 First part

First Part

Severe Congenital neutropenias (SCN) are a rare and complex group of diseases collected by the Severe Chronic Neutropenia International Registry organization. Here we report the most interesting information provided by the analysis of the registry data. SCN have been divided into congenital neutropenia (Kostman's Syndrome, Schwachman Diamond Syndrome, Glycogen storage disease type 1b and myelocathexis I), cyclic neutropenia and idiopathic neutropenias. Only patients with neutrophils 0.50 X109/L were enrolled. By the end of 1997, 531 patients were registered (249 with congenital neutropenia, 106 with the cyclic and 176 with the idiopathic form). 98% of the patients have been treated with Filgrastim (median dose 6.9 mcg/day) the mean treatment duration was 4.44 mcg/day year and about 10% of them reached the 10th year. The changes in the main hematological parameters from the pretreatment time to the 9th year of treatment were as follows:

  • Hemoglobin from 11.50 to 12.75 gr/dL

  • Absolute neutrophil count from 0.20 to 2.52 (X109/L)

  • White blood cell count from 4.00 to 6.40 (X109/L)

  • Platelet count from 339.00 to 208.90 (X109/L)

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