Hemophagocytic lymphohistiocytosis in total parenteral nutrition dependent children: Description of 5 cases and practical tips for management

Serena Pastore, Francesca Barbieri, Grazia Di Leo, Erica Valencic, Alberto Tommasini, Alessandro Ventura

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalJournal of Pediatric Hematology/Oncology
DOIs
Publication statusAccepted/In press - Jul 3 2013

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Hemophagocytic Lymphohistiocytosis
Total Parenteral Nutrition
Sepsis
Macrophage Activation
Metabolic Bone Diseases
Cholestasis
Infection
Treatment Failure
Liver Cirrhosis
Thrombosis
Catheters
Fats

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Pediatrics, Perinatology, and Child Health

Cite this

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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.",
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T2 - Description of 5 cases and practical tips for management

AU - Pastore, Serena

AU - Barbieri, Francesca

AU - Leo, Grazia Di

AU - Valencic, Erica

AU - Tommasini, Alberto

AU - Ventura, Alessandro

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