Korean J Pediatr Hematol Oncol.
1999 Apr;6(1):136-140.
Kostmann's Disease: A Case Report
- Affiliations
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- 1Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Puasn, Korea.
Abstract
- Kostmann's disease is a rare hematologic disease entity which is characterized by persistent neutropenia and recurrent bacterial infections since early in infancy. The authors experienced a 2-month-old female infant with disseminated bacterial skin infection since 3 weeks of age and persistent neutropenia. Diagnosis was made by neutropenia in the peripheral blood, maturation arrest of myeloid hemopoiesis at myelocyte stage in the finding of bone marrow aspiration and improving response of neutropenia with G-CSF administration. She had experienced 4 episodes of sepsis and one episode of acute otitis externa during follow-up until 14 months of age.
