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Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: What is the treatment?

Demirkol, D and Yildizdas, D and Bayrakci, B and Karapinar, B and Kendirli, T and Koroglu, TF and Dursun, O and Erkek, N and Gedik, H and Citak, A and Kesici, S and Karabocuoglu, M and Carcillo, JA (2012) Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: What is the treatment? Critical Care, 16 (2). ISSN 1364-8535

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Abstract

Introduction: Hyperferritinemia is associated with increased mortality in pediatric sepsis, multiple organ dysfunction syndrome (MODS), and critical illness. The International Histiocyte Society has recommended that children with hyperferritinemia and secondary hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) should be treated with the same immunosuppressant/cytotoxic therapies used to treat primary HLH. We hypothesized that patients with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS can be successfully treated with a less immunosuppressant approach than is recommended for primary HLH.Methods: We conducted a multi-center cohort study of children in Turkish Pediatric Intensive Care units with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS treated with less immunosuppression (plasma exchange and intravenous immunoglobulin or methyl prednisolone) or with the primary HLH protocol (plasma exchange and dexamethasone or cyclosporine A and/or etoposide). The primary outcome assessed was hospital survival.Results: Twenty-three children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS were enrolled (median ferritin = 6341 μg/dL, median number of organ failures = 5). Univariate and multivariate analyses demonstrated that use of plasma exchange and methyl prednisolone or intravenous immunoglobulin (n = 17, survival 100%) was associated with improved survival compared to plasma exchange and dexamethasone and/or cyclosporine and/or etoposide (n = 6, survival 50%) (P = 0.002).Conclusions: Children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS can be successfully treated with plasma exchange, intravenous immunoglobulin, and methylprednisone. Randomized trials are required to evaluate if the HLH-94 protocol is helpful or harmful compared to this less immune suppressive and cytotoxic approach in this specific population. © 2012 Demirkol et al.; licensee BioMed Central Ltd.


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Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Demirkol, D
Yildizdas, D
Bayrakci, B
Karapinar, B
Kendirli, T
Koroglu, TF
Dursun, O
Erkek, N
Gedik, H
Citak, A
Kesici, S
Karabocuoglu, M
Carcillo, JAjac22@pitt.eduJAC22
Date: 19 March 2012
Date Type: Publication
Journal or Publication Title: Critical Care
Volume: 16
Number: 2
DOI or Unique Handle: 10.1186/cc11256
Schools and Programs: School of Medicine > Pediatrics
Refereed: Yes
ISSN: 1364-8535
Date Deposited: 20 Oct 2016 18:23
Last Modified: 27 Aug 2019 20:55
URI: http://d-scholarship.pitt.edu/id/eprint/29941

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