Primary hemophagocytic lymphohistiocytosis in Turkish children |
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Authors: | Gürgey A Göğüş S Ozyürek E Aslan D Gümrük F Cetin M Yüce A Ceyhan M Seçmeer G Yetgin S Hiçsönmez G |
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Affiliation: | a Hacettepe University, Faculty of Medicine, Department of Pediatrics, Ihsan Dogramacı Children's Hospital, Ankara, Turkey. |
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Abstract: | 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. |
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Keywords: | Familial Lymphohistiocytosis Hemophagocytic Lymphohistiocytosis Sporadic Lymphohistiocytosis |
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