Kostmann's Disease: A Case Report.
- Author:
Cheol Gyu YOON
1
;
Jin Gyu JANG
;
Jong Beom SIN
;
Soon Yong LEE
Author Information
1. Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Puasn, Korea.
- Publication Type:Case Report
- Keywords:
Kostmann;
s disease;
G-CSF (Granulocyte colony-stimulating-factor);
Bone marrow transplantation
- MeSH:
Bacterial Infections;
Bone Marrow;
Bone Marrow Transplantation;
Diagnosis;
Female;
Follow-Up Studies;
Granulocyte Colony-Stimulating Factor;
Granulocyte Precursor Cells;
Hematologic Diseases;
Humans;
Infant;
Neutropenia;
Otitis Externa;
Sepsis;
Skin
- From:Korean Journal of Pediatric Hematology-Oncology
1999;6(1):136-140
- CountryRepublic of Korea
- Language:Korean
-
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.
