Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates

A Nationwide Survey in Japan

Nobuhiro Suzuki, Akira Morimoto, Shoichi Ohga, Kazuko Kudo, Yasushi Ishida, Eiichi Ishii

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

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.

Original languageEnglish
JournalJournal of Pediatrics
Volume155
Issue number2
DOIs
Publication statusPublished - Jan 1 2009

Fingerprint

Hemophagocytic Lymphohistiocytosis
Japan
Newborn Infant
Simplexvirus
Surveys and Questionnaires
Severe Combined Immunodeficiency
Hypertriglyceridemia
Hematology
Neutropenia
Premature Infants
Early Diagnosis
Fever
Survival Rate
Parturition
Pediatrics

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

Cite this

Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates : A Nationwide Survey in Japan. / Suzuki, Nobuhiro; Morimoto, Akira; Ohga, Shoichi; Kudo, Kazuko; Ishida, Yasushi; Ishii, Eiichi.

In: Journal of Pediatrics, Vol. 155, No. 2, 01.01.2009.

Research output: Contribution to journalArticle

Suzuki, Nobuhiro ; Morimoto, Akira ; Ohga, Shoichi ; Kudo, Kazuko ; Ishida, Yasushi ; Ishii, Eiichi. / Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates : A Nationwide Survey in Japan. In: Journal of Pediatrics. 2009 ; Vol. 155, No. 2.
@article{503ffe5514904ee9b93c5f0e9fb77191,
title = "Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates: A Nationwide Survey in Japan",
abstract = "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.",
author = "Nobuhiro Suzuki and Akira Morimoto and Shoichi Ohga and Kazuko Kudo and Yasushi Ishida and Eiichi Ishii",
year = "2009",
month = "1",
day = "1",
doi = "10.1016/j.jpeds.2009.02.050",
language = "English",
volume = "155",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "2",

}

TY - JOUR

T1 - Characteristics of Hemophagocytic Lymphohistiocytosis in Neonates

T2 - A Nationwide Survey in Japan

AU - Suzuki, Nobuhiro

AU - Morimoto, Akira

AU - Ohga, Shoichi

AU - Kudo, Kazuko

AU - Ishida, Yasushi

AU - Ishii, Eiichi

PY - 2009/1/1

Y1 - 2009/1/1

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.

UR - http://www.scopus.com/inward/record.url?scp=67651085515&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=67651085515&partnerID=8YFLogxK

U2 - 10.1016/j.jpeds.2009.02.050

DO - 10.1016/j.jpeds.2009.02.050

M3 - Article

VL - 155

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 2

ER -