RT Book, Section
A1 Hazen, Melissa M.
A2 Zaoutis, Lisa B.
A2 Chiang, Vincent W.
SR Print(0)
ID 1146122514
T1 Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
T2 Comprehensive Pediatric Hospital Medicine, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071829281
LK accesspediatrics.mhmedical.com/content.aspx?aid=1146122514
RD 2022/08/18
AB Hemophagocytic  lymphohistiocytosis  (HLH)  and  macrophage  activation  syndrome  (MAS)  are  clinically  related  life-threatening  immune  dysregulatory  processes  characterized  by  fever,  systemic  inflammation,  organomegaly,  coagulopathy,  and  hematologic  cytopenias.  Both  conditions  may  also  include  neurologic  symptoms,  often  accompanied  by  cerebrospinal  fluid  and  brain  imaging  abnormalities.  HLH  and  MAS  are  defined  histologically  by  the  phagocytosis  of  hematopoietic  cells  by  normal-appearing  macrophages.  This  hemophagocytosis  is  most  typically  seen  on  samples  from  bone  marrow  aspirates,  but  it  can  also  be  seen  in  the  spleen  and  in  other  lymphatic  tissue.