Hemophagocytic Lymphohistiocytosis: Advances in Pathophysiology, Diagnosis, and Treatment

被引：180
作者：
Chandrakasan, Shanmuganathan [1 ]
Filipovich, Alexandra H. [1 ]
机构：
[1] Cincinnati Childrens Hosp Med Ctr, Div Bone Marrow Transplantat & Immune Deficiency, Cincinnati, OH 45229 USA
来源：
JOURNAL OF PEDIATRICS | 2013年 / 163卷 / 05期
关键词：
MACROPHAGE ACTIVATION SYNDROME; LINKED LYMPHOPROLIFERATIVE DISEASE; STEM-CELL TRANSPLANTATION; XIAP DEFICIENCY; MUTATIONS; IMMUNODEFICIENCY; CHILDREN; PERFORIN; MUNC13-4; STXBP2;
D O I：
10.1016/j.jpeds.2013.06.053
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
HLH is a potentially fatal disorder of immune regulation. Depending on the severity of the underlying genetic defect, it can present across all age groups. Both pediatricians and subspecialists need to be aware of the atypical presentations of HLH. Colitis, bleeding tendency, and hypogammaglobulinemia are not uncommon in HLH. When evaluating patients with HLH, secondary HLH/MAS from underlying infections, rheumatologic disease, or malignant neoplasms should be considered as well. Many rapid diagnostic tests are available for diagnosing HLH. Increased awareness, early identification, and definitive management have improved outcomes. © 2013 Mosby Inc. All rights reserved.
引用
收藏
页码：1253 / 1259
页数：7
相关论文
共 60 条
[11]   Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis [J].
Haddad, E ;
Sulis, ML ;
Jabado, N ;
Blanche, S ;
Fischer, A ;
Tardieu, M .
BLOOD, 1997, 89 (03) :794-800
[12]   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
[13]   Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation [J].
Henter, JI ;
Samuelsson-Horne, A ;
Aricò, M ;
Egeler, RM ;
Elinder, G ;
Filipovich, AH ;
Gadner, H ;
Imashuku, S ;
Komp, D ;
Ladisch, S ;
Webb, D ;
Janka, G .
BLOOD, 2002, 100 (07) :2367-2373
[14]   HYPERCYTOKINEMIA IN FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS [J].
HENTER, JI ;
ELINDER, G ;
SODER, O ;
HANSSON, M ;
ANDERSSON, B ;
ANDERSSON, U .
BLOOD, 1991, 78 (11) :2918-2922
[15]   LIPOPROTEIN ALTERATIONS AND PLASMA-LIPOPROTEIN LIPASE REDUCTION IN FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS [J].
HENTER, JI ;
CARLSON, LA ;
SODER, O ;
NILSSONEHLE, P ;
ELINDER, G .
ACTA PAEDIATRICA SCANDINAVICA, 1991, 80 (6-7) :675-681
[16]   Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis [J].
Horne, AnnaCarin ;
Trottestam, Helena ;
Arico, Maurizio ;
Egeler, R. Maarten ;
Filipovich, Alexandra H. ;
Gadner, Helmut ;
Imashuku, Shinsaku ;
Ladisch, Stephan ;
Webb, David ;
Janka, Gritta ;
Henter, Jan-Inge .
BRITISH JOURNAL OF HAEMATOLOGY, 2008, 140 (03) :327-335
[17]   FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS [J].
JANKA, GE .
EUROPEAN JOURNAL OF PEDIATRICS, 1983, 140 (03) :221-230
[18]   Modern management of children with haemophagocytic lymphohistiocytosis [J].
Janka, GE ;
Schneider, EM .
BRITISH JOURNAL OF HAEMATOLOGY, 2004, 124 (01) :4-14
[19]   Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders [J].
Johnson, Theodore S. ;
Villanueva, Joyce ;
Filipovich, Alexandra H. ;
Marsh, Rebecca A. ;
Bleesing, Jack J. .
JOURNAL OF IMMUNOLOGICAL METHODS, 2011, 364 (1-2) :1-13
[20]   How I treat hemophagocytic lymphohistiocytosis [J].
Jordan, Michael B. ;
Allen, Carl E. ;
Weitzman, Sheila ;
Filipovich, Alexandra H. ;
McClain, Kenneth L. .
BLOOD, 2011, 118 (15) :4041-4052
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