Hemophagocytic Lymphohistiocytosis: Advances in Pathophysiology, Diagnosis, and Treatment

被引：238

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 [儿科学];

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

页码：1253 / 1259

页数：7

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