Kostmann's syndrome with chronic pneumonia and lymphocytosis: Effect of recombinant human G-CSF
Turkish Journal of Pediatrics, cilt.36, sa.1, ss.87-91, 1994 (Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 36 Sayı: 1
- Basım Tarihi: 1994
- Dergi Adı: Turkish Journal of Pediatrics
- Derginin Tarandığı İndeksler: Scopus
- Sayfa Sayıları: ss.87-91
- Anahtar Kelimeler: congenital agranulocytosis, congenital neutropenia, Kostmann's syndrome, recombinant human granulocyte-colony-stimulating factor
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Kostmann's syndrome is a congenital disorder characterized by impairment of myeloid differentiation in bone marrow with severe absolute neutropenia. A 17-month-old girl was admitted to the hospital with complaints of recurrent skin infections since birth and severe pneumonia of the right lung which had been resistant to antibiotics since the patient was eight months old. Anemia, severe neutropenia and maturational arrest of granulocytes at the myelocyte stage in bone marrow were detected. At the age of 20 months, a right pneumonectomy was performed because of resistant cystic infection. Postoperatively, she was diagnosed with Kostmann's syndrome. Recombinant human granulocyte-colony-stimulating factor (rhG-CSP) was administered intravenously at a dose of 3 μg/kg/day. gradually increasing to 60 μg/kg/day in sequential seven-day courses to obtain a neutrophil count of more than 500 cells/mm3. Absolute neutrophil counts increased to greater than 1000 cells/mm3 at a dose of 60 μg/kg/day, and at that time bone marrow aspiration revealed an increase in neutrophilic granulocytic precursors beyond the myelocyte stage. In order to maintain the neutrophil response, a dose of 20 μg/kg/day rhG-CSP subcutaneously was continued successfully. The patient has tolerated rhG-CSP treatment without complications, and infectious attacks have significantly decreased.