Primary hemophagocytic lymphohistiocytosis in Turkish children

Gurgey A., Gogus S., Ozyurek E., Aslan D., Gumruk F., Cetin M., ...Daha Fazla

PEDIATRIC HEMATOLOGY AND ONCOLOGY, cilt.20, sa.5, ss.367-371, 2003 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 20 Sayı: 5
  • Basım Tarihi: 2003
  • Doi Numarası: 10.1080/08880010390203891
  • Dergi Adı: PEDIATRIC HEMATOLOGY AND ONCOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.367-371
  • Anahtar Kelimeler: familial lymphohistiocytosis, hemophagocytic lymphohistiocytosis, sporadic lymphohistiocytosis, PERFORIN GENE, HYPERCYTOKINEMIA
  • Gazi Üniversitesi Adresli: Evet

Özet

Nineteen children with hemophagocytic lymphohistiocytosis (HLH) were studied in the Department of Pediatric Hematology, Hacettepe University. Patients were divided into two groups. Group 1 : Thirteen patients were classified as having a genetic etiology (7 familial, 6 presumed familial) on the basis of an affected sibling and consanguinity. There was a history of consanguineous marriage in 13 of the families. Seven of them had a history of a sibling with HLH. Group 2 : Six patients were diagnosed with sporadic HLH. The age at presentation for familial patients was 0.7-84 months (mean 21.9+/-24.9 months), and for sporadic cases it was 2.5-48 months (mean 22.7+/-19.8 months). The clinical and laboratory data of these two groups were similar at diagnosis. Thirteen cases were diagnosed premortem by bone marrow aspiration. Splenic biopsy was performed in 2 patients. Four patients were diagnosed by postmortem examination. Elevated LDH levels were found in all patients tested. No significant differences for clinical and laboratory data were found between the two groups.