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Validation of the HScore and the HLH-2004 diagnostic criteria for the diagnosis of hemophagocytic lymphohistiocytosis in a multicenter cohort.
Bilston, Lisa; Croden, Jennifer; Taparia, Minakshi; Karkhaneh, Mohammad; Grossman, Jennifer; Sun, Haowei Linda.
Afiliación
  • Bilston L; Department of Medicine, University of Calgary, Calgary, Canada.
  • Croden J; Department of Medicine, University of Alberta, Edmonton, Canada.
  • Taparia M; Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Canada.
  • Karkhaneh M; Institute of Health Economics, Edmonton, Canada.
  • Grossman J; Division of Hematology and Bone Marrow Transplantation, Department of Medicine, University of Calgary, Calgary, Canada.
  • Sun HL; Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Canada.
Eur J Haematol ; 109(2): 129-137, 2022 Aug.
Article en En | MEDLINE | ID: mdl-35434872
Timely diagnosis of hemophagocytic lymphohistiocytosis (HLH) is critical and relies on clinical judgment. The HLH-2004 criteria are commonly used diagnostic criteria, whereas HScore was recently developed for reactive HLH. OBJECTIVE: In this external validation study, we sought to compare the diagnostic accuracy of the HLH-2004 criteria and HScore and identify optimal cutoffs stratified by underlying etiology. METHODS: In this retrospective cohort of all hospitalized adults in Alberta, Canada, (1999-2019) who had ferritin >500 ng/ml and underwent either biopsies or soluble CD25 testing, we calculated the diagnostic accuracy of HLH-2004 and HScore for the overall population and different etiologies. RESULTS: Of 916 patients, 98 (11%) had HLH. HLH-2004 criteria ≥5 predicted HLH with a sensitivity of 91%, specificity of 93%, positive predictive value of 90%, and negative predictive value of 94% (c-statistic 92%). HScore ≥169 predicted HLH with better sensitivity (96%) but reduced specificity (71%), whereas the optimal cutoff ≥200 performed comparably to HLH-2004. HLH-2004 criteria outperformed HScore in most etiologies, whereas HScore improved sensitivity in inflammatory/autoimmune-HLH. The optimal cutoff of HScore was higher in hematopoietic cell transplant due to higher prevalence of fevers and cytopenias. CONCLUSION: HLH-2004 criteria and HScore demonstrated excellent discriminatory power in identifying HLH. HScore may improve diagnostic accuracy in autoimmune-HLH.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trasplante de Células Madre Hematopoyéticas / Linfohistiocitosis Hemofagocítica Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Humans País/Región como asunto: America do norte Idioma: En Revista: Eur J Haematol Asunto de la revista: HEMATOLOGIA Año: 2022 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trasplante de Células Madre Hematopoyéticas / Linfohistiocitosis Hemofagocítica Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Humans País/Región como asunto: America do norte Idioma: En Revista: Eur J Haematol Asunto de la revista: HEMATOLOGIA Año: 2022 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Reino Unido