YALÇIN ÇELİK, Bahar SALİHOĞLU, Sami HATİPOĞLU, Aysun KARADAĞ, Vefik ARICA

Kostmann sendromu: Vaka sunumu

Kostmann Sendromu, ağır konjenital nötropeni, kemik iliğinde miyelosit olgunlaşmasının promyelosit aşamasında duraklaması ile karakterize, mutlak nötrofil sayısının 200 /mm3'den az olması ile sonuçlanan bir hastalıktır. Hastalarda ciddi bakteriyel infeksiyonlar görülür. 1987'de rekombinan insan granulosit koloni stimulating faktörün (r-HuG-CSF) kullanılır olmasıyla nötropeninin prognozu ve hastaların yaşam kalitesi belirgin olarak değişmiştir. Bu yazıda sütçocukluğu döneminde başlayan tekrarlayan bakteriyel infeksiyonlar öyküsü olan 16 aylık erkek hasta Kostmann sendromunun klinik özelliklerini tartışmak amacıyla sunuldu.

Anahtar Kelimeler:

Bakteriyel enfeksiyonlar, Çocuk, Nötropeni

Kostmann syndrome: A case report

Kostmann Syndrome, severe congenital neutropenia, is characterized by an arrest in myeloid maturation at the promyelocyte stage of bone marrow, resulting in an absolute neutrophil count of less than 200/mm3. Patients suffer from severe bacterial infections. The availability of recombinant human granulocyte colony stimulating factor (r-HuG-CSF) in 1987 dramatically changed both the prognosis of neutropenia and the quality of life of patients. In this paper, we present 16-months-old boy with a history of recurrent bacterial infections starting in early infancy and discuss the clinical features of Kostmann Syndrome.

Keywords:

Bacterial Infections, Child, Neutropenia,

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