6533b857fe1ef96bd12b46c6
RESEARCH PRODUCT
Development and initial validation of the macrophage activation syndrome/primary hemophagocytic lymphohistiocytosis score, a diagnostic tool that differentiates primary hemophagocytic lymphohistiocytosis from macrophage activation syndrome
Francesca MinoiaFrancesca BovisSergio DavìAntonella InsalacoKai LehmbergSusan ShenoiSheila WeitzmanGraciela EspadaYi-jin GaoJordi AntonToshiyuki KitohOzgur KasapcopurHelga SannerRosa MerinoItziar AstigarragaMaria AlessioMichael JengVyacheslav ChasnykKim E. NicholsZeng HuasongCaifeng LiConcetta MicalizziNicolino RupertoAlberto MartiniRandy Q. CronAngelo RavelliAnnacarin HorneMario AbinunAmita AggarwalJonathan AkikusaSulaiman Al-mayoufMaria AlessioJordi AntonMaria Teresa ApazItziar AstigarragaTadej AvcinNuray Aktay AyazPatrizia BaroneBianca BicaIsabel BoltFrancesca BovisLuciana BredaVyacheslav ChasnykRolando CimazFabrizia CoronaRandy Q. CronRuben CutticaSergio DavìZane DavidsoneCarmen De CuntoJaime De InocencioErkan DemirkayaEli M. EisensteinSandra EncisoGraciela EspadaMichel FischbachMichael FroschRomina GallizziMaria Luz GamirYi-jin GaoThomas GriffinAlexei GromSoad HashadTeresa HennonJan-inge HenterAnnacarin HorneGerd HorneffZeng HuasongAdam HuberNorman IlowiteAntonella InsalacoMaka IoselianiMichael JengAgneza Marija KapovićOzgur KasapcopurRaju KhubchandaniToshiyuki KitohIsabelle Koné-pautSheila Knupp Feitosa De OliveiraBianca LattanziKai LehmbergLoredana LeporeCaifeng LiJeffrey M. LiptonSilvia Magni-manzoniDespoina MaritsiAlberto MartiniDeborah MccurdyRosa MerinoConcetta MicalizziPaivi MiettunenFrancesca MinoiaVelma MulaosmanovicKim E. NicholsSusan NielsenSeza OzenPriyankar PalSampath PrahaladAngelo RavelliDonato RiganteIngrida Rumba-rozenfeldeNicolino RupertoRicardo RussoClaudia Saad MagalhãesHelga SannerWafaa Mohamed Saad SewairiSusan ShenoiClovis Artur SilvaValda StanevichaGary SterbaKimo C. StineGordana SusicFlavio SztajnbokSyuji TakeiRalf TrauzeddelElena TsitsamiErbil UnsalYosef UzielOlga VougioukaCarol A. WallaceLehn WeaverJennifer E. WeissSheila WeitzmanCarine WoutersNico WulffraatMabruka ZletniAricò MaurizioR. Maarten EgelerAlexandra H. FilipovichHelmut GadnerShinsaku ImashukuGritta JankaStephan LadischKen L. McclainDavid Webbsubject
Male0301 basic medicineHemophagocyticLogistic regressionPediatricshemophagocytic syndrome0302 clinical medicine*diagnostic scoreDiagnosisMedicineCutoffChildprimary hemophagocytic lymphohistiocytosiLymphohistiocytosiseducation.field_of_studyprimary hemophagocytic lymphohistiocytosisPerinatology and Child Healthdiagnostic scoreQuartileSettore MED/38 - PEDIATRIA GENERALE E SPECIALISTICAMacrophage activation syndromeChild Preschool*macrophage activation syndromeAbsolute neutrophil countFemale*primary hemophagocytic lymphohistiocytosisHumanmedicine.medical_specialtyAdolescentPopulationLymphohistiocytosis HemophagocyticDiagnosis Differential03 medical and health sciencesInternal medicineHumansPreschooleducation030203 arthritis & rheumatologyReceiver operating characteristicbusiness.industryInfantReproducibility of Resultsmedicine.diseaseSurgery030104 developmental biologydiagnostic score; hemophagocytic syndrome; macrophage activation syndrome; primary hemophagocytic lymphohistiocytosis; Adolescent; Child; Child Preschool; Diagnosis Differential; Female; Humans; Infant; Lymphohistiocytosis Hemophagocytic; Macrophage Activation Syndrome; Male; Reproducibility of Results; Pediatrics Perinatology and Child HealthMacrophage activation syndromeDifferentialPediatrics Perinatology and Child Health*hemophagocytic syndromeDifferential diagnosisbusinessdescription
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample. Variables that entered the best fitted model of logistic regression were assigned a score, based on their statistical weight. The MAS/HLH (MH) score was made up with the individual scores of selected variables. The cutoff in the MH score that discriminated pHLH from MAS best was calculated by means of receiver operating characteristic curve analysis. Score performance was examined in both developmental and validation samples. RESULTS: Six variables composed the MH score: age at onset, neutrophil count, fibrinogen, splenomegaly, platelet count, and hemoglobin. The MH score ranged from 0 to 123, and its median value was 97 (1st-3rd quartile 75-123) and 12 (1st-3rd quartile 11-34) in pHLH and MAS, respectively. The probability of a diagnosis of pHLH ranged from 99% for a score of =123. A cutoff value of =60 revealed the best performance in discriminating pHLH from MAS. CONCLUSION: The MH score is a powerful tool that may aid practitioners to identify patients who are more likely to have pHLH and, thus, could be prioritized for functional and genetic testing.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-10-01 |