Primary Hemophagocytic Lymphohistiocytosis in Turkish Children

• Published in: Pediatric hematology and oncology Vol. 20; no. 5; pp. 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: Journal Article
• Language: English
• Published: Philadelphia, PA Informa UK Ltd 2003; Taylor & Francis
• 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; Asia; Turkey; Human; Hemophagocytic lymphohistiocytosis; Symptomatology; sporadic lymphohistiocytosis; familial lymphohistiocytosis; Diagnosis; Child; Epidemiology; Genetic disease
• ISSN: 0888-0018; 1521-0669
• DOI: 10.1080/08880010390203891

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.