Ｋｏｓｔｍａｎｎ型先天性好中球減少症の兄弟例

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  • Two Siblings with Infantile Genetic Agranulocytosis (Kostmann Type).

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Two siblings, a 2-year-3-month-old boy and a 14-day-old boy, who were afflicted with infantile genetic agranulocytosis (Kostmann Type) received different methods of treatment with rhG-CSF. Each patient was closely observed so as to monitor any differences in the response to the treatments. Specifically we looked at changes in their clinical symptoms and subsequent levels of neutrophils following their treatments. The rhG-CSF was administered to the patients in the following ways continuous intravenous infusions over a period of 24 hours, intravenous infusion for an hour, and subcutaneous injection. In both patients we observed rapid appearance of neutrophils and marked improvement of their clinical symptoms. The patient who received continuous infusion of rhG-CSF over a 24-hour time span showed an increase in the number of neutrophils and an improvement in CRP. We conclude that further study regarding the proper administration of rhG-CSF should focus on the relationship between the number of neutrophils and the concentration of rhG-CSF in the serum at the time of each administration.

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