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

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dc.contributor.authorDemirkol, Demet
dc.contributor.authorYildizdas, Dincer
dc.contributor.authorBayrakci, Benan
dc.contributor.authorKarapinar, Bulent
dc.contributor.authorKendirli, Tanil
dc.contributor.authorKoroglu, Tolga F.
dc.contributor.authorDursun, Oguz
dc.contributor.authorErkek, Nilgun
dc.contributor.authorGedik, Hakan
dc.contributor.authorCitak, Agop
dc.contributor.authorKesici, Selman
dc.contributor.authorKarabocuoglu, Metin
dc.contributor.authorCarcillo, Joseph A.
dc.date.accessioned2019-10-27T21:33:47Z
dc.date.available2019-10-27T21:33:47Z
dc.date.issued2012
dc.departmentEge Üniversitesien_US
dc.description.abstractIntroduction: 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 mu g/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.en_US
dc.identifier.doi10.1186/cc11256en_US
dc.identifier.issn1466-609X
dc.identifier.issn1364-8535
dc.identifier.issue2en_US
dc.identifier.pmid22715953en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1186/cc11256
dc.identifier.urihttps://hdl.handle.net/11454/45405
dc.identifier.volume16en_US
dc.identifier.wosWOS:000313196800018en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherBmcen_US
dc.relation.ispartofCritical Careen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleHyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?en_US
dc.typeArticleen_US

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