Hemophagocytic Lymphohistiocytosis in Children With Chronic Granulomatous Disease

被引：34

作者：

Parekh, Chintan ^{[1
]}

Hofstra, Thomas ^{[1
]}

Church, Joseph A. ^{[2
]}

Coates, Thomas D. ^{[1
]}

机构：

[1] Childrens Hosp Los Angeles, Div Pediat Hematol Oncol, Los Angeles, CA 90027 USA

[2] Childrens Hosp Los Angeles, Div Clin Immunol & Allergy, Los Angeles, CA 90027 USA

来源：

PEDIATRIC BLOOD & CANCER | 2011年 / 56卷 / 03期

关键词：

children; chronic granulomatous disease; hemophagocytosis; INFECTION;

D O I：

10.1002/pbc.22830

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, cytopenias, splenomegaly, and hemophagocytosis by macrophages activated by high cytokine levels. Chronic granulomatous disease (CGD) is characterized by recurrent infections, hyperinflammation, and excessive cytokine release. This may predispose patients with CGD to developing HLH during an infection. We conducted a retrospective review of patients with CGD, treated at our institution between 1999 and 2008. Three out of 17 patients developed HLH. Patients with CGD may be at increased risk for developing HLH. Remission of HLH was achieved after treatment with antimicrobials, steroids, and intravenous immunoglobulin This approach to treatment appears to be effective. Pediatr Blood Cancer. Pediatr Blood Cancer 2011;56:460-462. (C) 2010 Wiley-Liss, Inc.

引用

页码：460 / 462

页数：3

共 10 条

[1] Secondary hemophagocytic lymphohistiocytosis and severe sepsis/systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation [J].

Castillo, Leticia ;

Carcillo, Joseph .

PEDIATRIC CRITICAL CARE MEDICINE, 2009, 10 (03) :387-392

[2] Hemophagocytic syndromes and infection [J].

Fisman, DN .

EMERGING INFECTIOUS DISEASES, 2000, 6 (06) :601-608

[3] HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [J].

Henter, Jan-Inge ;

Horne, AnnaCarin ;

Arico, Maurizio ;

Egeler, R. Maarten ;

Filipovich, Alexandra H. ;

Imashuku, Shinsaku ;

Ladisch, Stephan ;

McClain, Ken ;

Webb, David ;

Winiarski, Jacek ;

Janka, Gritta .

PEDIATRIC BLOOD & CANCER, 2007, 48 (02) :124-131

[4] Nationwide survey of hemophagocytic lymphohistiocytosis in Japan [J].

Ishii, Eiichi ;

Ohga, Shouichi ;

Imashuku, Shinsaku ;

Yasukawa, Masaki ;

Tsuda, Hiroyuki ;

Miura, Ikuo ;

Yamamoto, Ken ;

Horiuchi, Hisanori ;

Takada, Kenzo ;

Ohshima, Koichi ;

Nakamura, Shigeo ;

Kinukawa, Naoko ;

Oshimi, Kazuo ;

Kawa, Keisei .

INTERNATIONAL JOURNAL OF HEMATOLOGY, 2007, 86 (01) :58-65

[5] Infection- and malignancy-associated hemophagocytic syndromes - Secondary hemophagocytic lymphohistiocytosis [J].

Janka, G ;

Imashuku, S ;

Elinder, G ;

Schneider, M ;

Henter, JI .

HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA, 1998, 12 (02) :435-+

[6] Hemophagocytic syndromes [J].

Janka, Gritta E. .

BLOOD REVIEWS, 2007, 21 (05) :245-253

[7] Hyperinflammation in chronic granulomatous disease and anti-inflammatory role of the phagocyte NADPH oxidase [J].

Schaeppi, Michela G. ;

Jaquet, Vincent ;

Belli, Dominique C. ;

Krause, Karl-Heinz .

SEMINARS IN IMMUNOPATHOLOGY, 2008, 30 (03) :255-271

[8] Modern management of chronic granulomatous disease [J].

Seger, Reinhard A. .

BRITISH JOURNAL OF HAEMATOLOGY, 2008, 140 (03) :255-266

[9] Hemophagocytic syndrome and Burkholderia cepacia splenic microabscesses in a child with chronic granulomatolis disease [J].

Sirinavin, S ;

Techasaensiri, C ;

Pakakasama, S ;

Vorachit, M ;

Pornkul, R ;

Wacharasin, R .

PEDIATRIC INFECTIOUS DISEASE JOURNAL, 2004, 23 (09) :882-884

[10] Fatal Hemophagocytic Lymphohistiocytosis in X-Linked Chronic Granulomatous Disease Associated With a Perforin Gene Variant [J].

van Montfrans, Joris A. ;

Rudd, Eva ;

van de Corput, Lisette ;

Henter, Jan-Inge ;

Nikkels, Peter ;

Wulffraat, Nico ;

Boelens, Jaap J. .

PEDIATRIC BLOOD & CANCER, 2009, 52 (04) :527-529

← 1 →