A case of infantile genetic agranulocytosis (Kostmann type) with increasing in the numbers of neutrophils by rhG-CSF administration.

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  • ｒｈＧ‐ＣＳＦ投与により好中球増加がみられたＫｏｓｔｍａｎｎ型先天性好中球減少症の一例

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Abstract

This case illustrates that treatment with rhG-CSF was effective for increasing the numbers of neutrophils and for infectious disease in a patient with infantile genetic agranulocytosis (Kostmann type). The patient is a 2 years and 3 months old boy who was diagnosed as infantile genetic agranulocytosis (Kostmann type) at the age of 4 months. He was admitted to our hospital for pneumonia at the age of 2 years and 3 months. Bone marrow aspiration on admission revealed a complete absence of neutrophils. At day 38, rhG-CSF (KRN8601) therapy was started at the dose of 400 μg/m2 with 30 min continuous infusion. At day 13 after treatment, WBC reached 7, 600/μl with 13 percent band and 18 percent segmented neutrophils. At day 21, bone marrow showed increased granulocytes. Nuclear cell count was 11.6 × 104/μl, granulocytes was 17.9 percent with 2.1 percent band and 2.2 percent segmented neutrophils, and erythrocytes was 15.8 percent. Within days of the start of rhG-CSF therapy, the inflammatory findings were improved markedly. We conclude that treatment with rhG-CSF leads to increase in the numbers of neutrophils temporarily in a patient with infantile genetic agranulocytosis (Kostmann type), and it is effective for the treatment of infectious disease in that patient.

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