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Hemophagocytic Lymphohistiocytosis in Total Parenteral Nutrition Dependent Children: Description of 5 Cases and Practical Tips for Management.

PASTORE, SERENA
•
Barbieri F
•
Leo GD
altro
VENTURA, ALESSANDRO
2014
  • journal article

Periodico
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
Abstract
Although total parenteral nutrition (TPN) is mandatory in children with intestinal failure, this treatment is not risk free. The main complications of TPN include catheter-related sepsis, thrombosis, hepatic cholestasis and cirrhosis, metabolic bone disease, and, rarely, reactive hemophagocytic lymphohistiocytosis (HLH). The pathogenesis of HLH in patients with TPN is not known, although some authors hypothesized that it can result from the activation of macrophages because of "fat overload." We reported 5 cases of HLH that occurred in patients with 4 different underlying disorders, all requiring TPN for a long term. In our series, an underlying immunological defect or a serious infection (sepsis) can have triggered HLH. Therefore, it could be reasonable to hypothesize that besides TPN in itself, minor immune defects and infections may act together by overcoming a threshold of immune stimulation, which ultimately leads to HLH.
DOI
10.1097/MPH.0b013e31829f381b
WOS
WOS:000343045300009
Archivio
http://hdl.handle.net/11368/2691804
info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-84910134463
Diritti
metadata only access
Soggetti
  • parenteral nutrition

  • hemophagocytic lympho...

Scopus© citazioni
4
Data di acquisizione
Jun 14, 2022
Vedi dettagli
Web of Science© citazioni
2
Data di acquisizione
Mar 26, 2024
Visualizzazioni
19
Data di acquisizione
Apr 19, 2024
Vedi dettagli
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