Primary Hemophagocytic Lymphohistiocytosis in Turkish Children

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...

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Bibliographic Details
Published in:Pediatric hematology and oncology 2003, Vol.20 (5), p.367-371
Main Authors: Gürgey, A., Gö ü, S., Özyürek, E., Aslan, D., Gümrük, F., Çetin, M., Yüce, A., Ceyhan, M., Seçmeer, G., Yetgin, S., Hiçsönmez, G.
Format: Article
Language:English
Subjects:
Biological and medical sciences
Bone Marrow - pathology
Child
Child, Preschool
Consanguinity
Familial Lymphohistiocytosis
Hematologic and hematopoietic diseases
Hemophagocytic Lymphohistiocytosis
Histiocytosis, Non-Langerhans-Cell - diagnosis
Histiocytosis, Non-Langerhans-Cell - epidemiology
Histiocytosis, Non-Langerhans-Cell - genetics
Histiocytosis, Non-Langerhans-Cell - pathology
Humans
Hydro-Lyases - blood
Infant
Infant, Newborn
Medical sciences
Membrane Glycoproteins - genetics
Other diseases. Hematologic involvement in other diseases
Perforin
Pore Forming Cytotoxic Proteins
Spleen - pathology
Sporadic Lymphohistiocytosis
Turkey - epidemiology
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Summary: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 &#45 24.9 months), and for sporadic cases it was 2.5-48 months (mean 22.7 &#45 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.
ISSN:0888-0018
1521-0669
DOI:10.1080/08880010390203891