PMID: 12775534May 31, 2003Paper

Primary hemophagocytic lymphohistiocytosis in Turkish children

Pediatric Hematology and Oncology
A GürgeyG Hiçsönmez

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.

Citations

Dec 21, 2012·Pathology·Bryce Higa, Milind Velankar
Jan 11, 2014·Journal of Intensive Care Medicine·Zuzana Tothova, Nancy Berliner
Jul 22, 2017·Journal of Pediatric Hematology/oncology·Sevgen Tanir BasaranogluMehmet Ceyhan
Jan 10, 2009·Journal of Pediatric Hematology/oncology·Aytemiz GurgeyMurat Yurdakok
Dec 24, 2005·The Pediatric Infectious Disease Journal·Aytemiz GurgeyFatma Gumruk
Mar 12, 2015·Blood·Alison M Schram, Nancy Berliner
Sep 19, 2019·AJR. American Journal of Roentgenology·Alice C ShiehNikhil H Ramaiya
May 13, 2009·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Bülent KarapinarKaan Kvakli
Feb 10, 2011·Clinical and Experimental Immunology·C GholamH B Gaspar
Apr 23, 2015·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Zühre KayaTurkiz Gursel
May 19, 2012·Pediatric Blood & Cancer·Fatih KardasMustafa Kendirci
Jul 16, 2013·Pediatrics International : Official Journal of the Japan Pediatric Society·Jeong-Min RyuJong Jin Seo
Jul 18, 2020·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Parisa AmirifarReza Yazdani

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