PMID: 9544223Apr 17, 1998Paper

Clinical and epidemiologic studies of familial hemophagocytic lymphohistiocytosis in Japan. Japan LCH Study Group

Medical and Pediatric Oncology
Eiichi IshiiS Miyazaki

Abstract

The etiology of familial hemophagocytic lymphohistiocytosis (FHL), which is characterized by fever, hepatosplenomegaly, pancytopenia, and coagulopathy, remains unknown. We analyzed 43 FHL patients, all with affected siblings, in 18 families who were identified during the period 1986-1995 in Japan. The presence of consanguinity was evident in two families (11%). The majority of families lived in western Japan, where the frequency of consanguineous marriage is high. The incidence of FHL was significantly higher in the western island, Kyushu, than in other areas. The segregation ratio calculated for these families was 0.35 by the Weinberg proband method, showing the autosomal-recessive inheritance of the disease. Since the diagnosis of an FHL patient without affected siblings (sporadic case) is quite difficult, we calculated the possible number of sporadic cases; approximately 122 patients could be identified as sporadic FHL cases during the same period in Japan. Most of the clinical and laboratory findings were not distinguishable from those of other types of lymphohistiocytosis. However, atypical lymphoid cells with azurophilic granules in peripheral blood were observed in half of the patients at diagnosis, suggesting the clinic...Continue Reading

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Citations

Jan 18, 2006·European Journal of Pediatrics·Hiroshi MizumotoEiichi Ishii
Jul 18, 2020·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Parisa AmirifarReza Yazdani
Feb 14, 2002·British Journal of Haematology·Naohiro SugaToshiro Hara
Feb 10, 2011·Clinical and Experimental Immunology·C GholamH B Gaspar
Apr 16, 2003·British Journal of Haematology·Nobuyuki YoshidaMasaki Yasukawa
May 26, 2006·Pediatric Blood & Cancer·Vasiliki Vlacha, Gavriella Feketea
Jan 18, 2013·BMC Infectious Diseases·Valentina AnsuiniSusanna Esposito
Apr 24, 2020·Journal of Pediatric Hematology/oncology·Pinar G CetinkayaIlhan Tezcan
Feb 2, 2006·Annals of Medicine·James W Verbsky, William J Grossman
May 22, 2013·American Journal of Clinical Pathology·Flavia G N Rosado, Annette S Kim
Feb 14, 2016·Indian Journal of Pediatrics·Manisha MadkaikarMukesh Desai
Nov 11, 2016·Archives of Disease in Childhood·Ethan S SenAthimalaipet V Ramanan
Feb 4, 2010·Reviews in Medical Virology·Nadine Rouphael MaakarounHelmut Albrecht
Aug 21, 2002·Obstetrical & Gynecological Survey·Caroline M de Costa
Mar 12, 2015·Blood·Alison M Schram, Nancy Berliner

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