Search results for "Hemophagocytic syndrome"

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Development and initial validation of the macrophage activation syndrome/primary hemophagocytic lymphohistiocytosis score, a diagnostic tool that dif…

2017

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 i…

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 diagnosisbusiness
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Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

2015

Objective.To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.Methods.International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.Results.A total of 362 patients were incl…

MaleHEMOPHAGOCYTIC SYNDROMESInternationalityDatabases FactualHepatosplenomegalyJuvenileComorbidityHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSISSeverity of Illness IndexHEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HEMOPHAGOCYTIC SYNDROMES; MACROPHAGE ACTIVATION SYNDROME; SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS; Adolescent; Age Distribution; Arthritis Juvenile; Child; Child Preschool; Cohort Studies; Comorbidity; Databases Factual; Female; Humans; Internationality; Macrophage Activation Syndrome; Male; Multivariate Analysis; Prevalence; Prognosis; Retrospective Studies; Severity of Illness Index; Sex Distribution; Survival Analysis; Immunology and Allergy; Rheumatology; ImmunologyCohort StudiesPrevalenceImmunology and AllergyChildMacrophage Activation SyndromePrognosisChild PreschoolFemaleHemophagocytosismedicine.symptommedicine.medical_specialtyAdolescentSYSTEMIC JUVENILE IDIOPATHIC ARTHRITISImmunologyHemophagocytic Lymphohistiocytosis Hemophagocytic Syndromes Macrophage activation syndromes Systemic Juvenile Idiopathic Arthritis Adolescent Age Distribution Arthritis Juvenile Child Child Preschool Cohort Studies Comorbidity Databases Factual Female Humans Internationality Macrophage Activation Syndrome Male Multivariate Analysis Prevalence Prognosis Retrospective Studies Severity of Illness Index Sex Distribution Survival Analysis Immunology and Allergy Rheumatology ImmunologyDatabasesAge DistributionRheumatologyInternal medicineSeverity of illnessmedicineHumansSex DistributionPreschoolFactualRetrospective StudiesHemophagocytic lymphohistiocytosisbusiness.industryArthritisRetrospective cohort studymedicine.diseaseSurvival AnalysisComorbidityArthritis JuvenileRheumatologyMacrophage activation syndromeMultivariate AnalysisImmunologyMacrophage activation syndromesbusinessThe Journal of Rheumatology
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Hemophagocytic syndrome in a patient with disseminated tuberculosis: a case report

2018

Hemophagocytic syndrome (HS) is a rare disorder of the immune system. It is characterized by fever, lymphadenopathy, hepatosplenomegaly, cytopenia and hyperferritinemia. The cause differs in each country suggesting a specific genetic background and epidemiology of infections, and it can be associated with malignant diseases. A rare cause of HS is tuberculosis (TB), we describe a case of HS associated with disseminated Mycobacterium tuberculosis (MT) infection in a patient from Sudan. He presented diarrhea, fever, pancytopenia, thickened and dilated bowel loops and lymph nodes enlargement at ultrasound and computed tomography scan. A bone marrow biopsy performed to rule out a lymphoma reveal…

Pathologymedicine.medical_specialtySettore MED/09 - Medicina InternaTuberculosisHepatosplenomegalyMycobacterium tuberculosis.lcsh:MedicineMycobacterium tuberculosiMycobacterium tuberculosisBiopsymedicineHemophagocytic lymphohistiocytosis disseminated tuberculosis; Hemophagocytic syndrome; Mycobacterium tuberculosis; Medicine (all)hemophagocytic lymphohistiocytosis disseminated tuberculosisCytopeniabiologymedicine.diagnostic_testbusiness.industryMedicine (all)lcsh:RGeneral Medicinebiology.organism_classificationmedicine.diseasePancytopeniaLymphomaBronchoalveolar lavageHemophagocytic lymphohistiocytosis disseminated tuberculosimedicine.symptombusinessHemophagocytic syndromeItalian Journal of Medicine
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