Prognostic Factors and Long-Term Outcome in 52 Turkish Children With Hemophagocytic Lymphohistiocytosis

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dc.contributor.authorKaya, Zuhre
dc.contributor.authorBay, Ali
dc.contributor.authorAlbayrak, Meryem
dc.contributor.authorKocak, Ulker
dc.contributor.authorYenicesu, Idil
dc.contributor.authorGursel, Turkiz
dc.date.accessioned2020-06-25T18:13:06Z
dc.date.available2020-06-25T18:13:06Z
dc.date.issued2015
dc.departmentKırıkkale Üniversitesi
dc.descriptionAlbayrak, Meryem/0000-0003-2711-5150
dc.description.abstractObjectives: Hemophagocytic lymphohistiocytosis is a syndrome of pathologic immune activation that shares similar clinical and laboratory phenotypes with severe sepsis. Recent studies led to better recognition of hemophagocytic lymphohistiocytosis by clinicians, but no consensus exists on the criteria for high-risk patients. Design: We retrospectively reviewed the medical records of patients diagnosed with hemophagocytic lymphohistiocytosis to analyze the risk factors associated with poor outcome. Setting: Pediatric intensive care and hematology units of three tertiary hospitals in Turkey. Participants: Fifty-two children with hemophagocytic lymphohistiocytosis. Interventions: None. Measurement and Main Results: There were a total of 52 children meeting the diagnostic criteria of Histiocytic Society. Of them, 28 (54%) had a primary hemophagocytic lymphohistiocytosis. Mutation studies were performed in 18 of 28 patients (65%). Fourteen of them had PRF1, STX11, STXBP2, and UNC13D mutations, and four had Rab27a and LYST mutations. The remaining 24 patients (46%) were defined as having secondary hemophagocytic lymphohistiocytosis. Twenty-one of them had infection-associated hemophagocytic lymphohistiocytosis, and three had lysinuric protein intolerance. The mortality rate was significantly higher in primary hemophagocytic lymphohistiocytosis (64%) than in secondary hemophagocytic lymphohistiocytosis (16%) (p < 0.05). There were no significant differences for survival rate between hemophagocytic lymphohistiocytosis 94 (44%) and hemophagocytic lymphohistiocytosis 2004 (64%) protocols (p > 0.05). Age below 2 years, hyperferritinemia, thrombocytopenia, high disseminated intravascular coagulation score at diagnosis, and no clinical response at 2 weeks of treatment were independent prognostic factors for poor prognosis. Conclusions: Our data suggest that disseminated intravascular coagulation score greater than or equal to 5 can be used in the definition of high-risk patients. Early recognition of poor risk factors has important prognostic and therapeutic implications.en_US
dc.identifier.citationclosedAccessen_US
dc.identifier.doi10.1097/PCC.0000000000000449
dc.identifier.endpageE173en_US
dc.identifier.issn1529-7535
dc.identifier.issn1947-3893
dc.identifier.issue6en_US
dc.identifier.pmid25901543
dc.identifier.scopus2-s2.0-84937711821
dc.identifier.scopusqualityQ1
dc.identifier.startpageE165en_US
dc.identifier.urihttps://doi.org/10.1097/PCC.0000000000000449
dc.identifier.urihttps://hdl.handle.net/20.500.12587/6126
dc.identifier.volume16en_US
dc.identifier.wosWOS:000358289900002
dc.identifier.wosqualityQ1
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherLippincott Williams & Wilkinsen_US
dc.relation.ispartofPediatric Critical Care Medicine
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectchildrenen_US
dc.subjecthemophagocytic syndromeen_US
dc.subjectprognosisen_US
dc.titlePrognostic Factors and Long-Term Outcome in 52 Turkish Children With Hemophagocytic Lymphohistiocytosisen_US
dc.typeArticle

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