TY - JOUR
T1 - Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates
T2 - A Nationwide Survey in Japan
AU - Suzuki, Nobuhiro
AU - Morimoto, Akira
AU - Ohga, Shouichi
AU - Kudo, Kazuko
AU - Ishida, Yasushi
AU - Ishii, Eiichi
PY - 2009/8
Y1 - 2009/8
N2 - Objective: To assess the etiology, prognosis, and appropriate treatment of hemophagocytic lymphohistiocytosis (HLH) in neonates. Study design: We collected information on neonates in whom HLH was diagnosed between 1997 and 2007 from participating members of the Japanese Society of Pediatric Hematology. Results: HLH was diagnosed in 20 patients within 4 weeks after birth. Of the diagnostic criteria for HLH-2004, the incidence of fever was quite low in preterm infants, and hypertriglyceridemia and neutropenia were uncommon. Familial HLH (n = 6) or severe combined immunodeficiency-associated HLH (n = 1) was diagnosed in 7 patients, and 2 of them have survived. Herpes simplex virus-associated HLH was diagnosed in 6 patients, and 2 of them have survived. The overall survival rate for the 20 patients was 40%. Conclusions: HLH is rare in neonates and has a poor prognosis. Early diagnosis and immediate treatment are required when considering the possibility of herpes simplex virus-associated or familial HLH.
AB - Objective: To assess the etiology, prognosis, and appropriate treatment of hemophagocytic lymphohistiocytosis (HLH) in neonates. Study design: We collected information on neonates in whom HLH was diagnosed between 1997 and 2007 from participating members of the Japanese Society of Pediatric Hematology. Results: HLH was diagnosed in 20 patients within 4 weeks after birth. Of the diagnostic criteria for HLH-2004, the incidence of fever was quite low in preterm infants, and hypertriglyceridemia and neutropenia were uncommon. Familial HLH (n = 6) or severe combined immunodeficiency-associated HLH (n = 1) was diagnosed in 7 patients, and 2 of them have survived. Herpes simplex virus-associated HLH was diagnosed in 6 patients, and 2 of them have survived. The overall survival rate for the 20 patients was 40%. Conclusions: HLH is rare in neonates and has a poor prognosis. Early diagnosis and immediate treatment are required when considering the possibility of herpes simplex virus-associated or familial HLH.
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U2 - 10.1016/j.jpeds.2009.02.050
DO - 10.1016/j.jpeds.2009.02.050
M3 - Article
C2 - 19446847
AN - SCOPUS:67651085515
SN - 0022-3476
VL - 155
SP - 235-238.e1
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 2
ER -