| A Case Report Kostmann Syndrome. |
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Han Wook Yoo, Son Moon Shin, Hyo Seop Ahn, Yong Choi, Chang Yee Hong |
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Department of Pediatrics, College of Medicine、Seoul National University, Seoul, Korea |
| Kostmann 증후군 1례 |
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유한욱, 신손문, 안효섭, 최 용, 홍창의 |
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서울대학교 의과대학 소아과학교실 |
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| Abstract |
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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 25 months, follow up period,
she showed persistent neutropenia, monocytosis and eosinophilia in peripheral blood. Maturation
arrest in early myelocytic series was remarkable in bone marrow. Neutropenia was not res-
ponsive to epinephrine stimulation test. Anti-granulocyte antibody was not detected.
Immunoglobulin quantitation revealed no abnormality. Peripheral neutrophil counts of parents
were normal. Family history was not remarkable.
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| Key Words:
Kostmann syndrome (Infantile Genetic Agranulocytosis). |
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