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

C. Wouters | T. Griffin | K. Nichols | D. McCurdy | S. Ozen | V. Chasnyk | S. Prahalad | A. Martini | J. Lipton | R. Cron | H. Gadner | R. Cimaz | A. Huber | M. Aricò | D. Rigante | M. Fischbach | S. Nielsen | A. Filipovich | M. Frosch | A. Horne | J. Henter | N. Wulffraat | S. Magni-Manzoni | J. Antón | I. Koné-Paut | T. Avčin | K. McClain | N. Ruperto | S. Takei | Y. Uziel | C. Silva | F. Sztajnbok | L. Lepore | C. Wallace | C. Micalizzi | M. Jeng | A. Aggarwal | N. Ilowite | A. Grom | O. Kasapcopur | N. Ayaz | E. Unsal | E. Demirkaya | A. Insalaco | J. Akikusa | E. Eisenstein | J. Weiss | T. Hennon | S. Weitzman | S. Ladisch | F. Bovis | F. Corona | M. Egeler | D. Webb | P. Barone | M. Abinun | S. Imashuku | K. Stine | S. Al‐Mayouf | E. Tsitsami | P. Miettunen | A. Ravelli | L. Breda | I. Bolt | G. Sušić | R. Merino | G. Horneff | V. Staņēvicha | O. Vougiouka | J. de Inocencio | G. Janka | T. Kitoh | R. Khubchandani | C. D. De Cunto | G. Espada | R. Russo | M. Alessio | Sheila Knupp Feitosa de Oliveira | C. Magalhaes | M. Apaz | P. Pal | B. Lattanzi | F. Minoia | R. Gallizzi | S. Davì | K. Lehmberg | G. Sterba | Ingrida Rumba-Rozenfelde | L. Weaver | R. Cuttica | I. Astigarraga | H. Sanner | M. Ioseliani | R. Trauzeddel | S. Shenoi | D. Maritsi | S. Enciso | Cai-feng Li | M. Gamir | M. Zletni | Z. Huasong | Wafaa Sewairi | V. Mulaosmanović | I. Rumba-Rozenfelde | Z. Dāvidsone | Soad Hashad | Yihong Gao | Z. Davidsone | B. Bica | Agneza Marija Kapović | Wafaa Mohamed Saad Sewairi | Caifeng Li | Soad S Hashad | Lehn K. Weaver

[1]  R. Schneider,et al.  Systemic Juvenile Idiopathic Arthritis. , 2018, Pediatric clinics of North America.

[2]  D. Giustini,et al.  Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. , 2016, Blood reviews.

[3]  A. Fischer,et al.  Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. , 2016, Blood.

[4]  Lauren B. Smith,et al.  Whole-Exome Sequencing Reveals Mutations in Genes Linked to Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Fatal Cases of H1N1 Influenza. , 2016, The Journal of infectious diseases.

[5]  Hao Li,et al.  A Heterozygous RAB27A Mutation Associated with Delayed Cytolytic Granule Polarization and Hemophagocytic Lymphohistiocytosis , 2016, The Journal of Immunology.

[6]  C. Wouters,et al.  2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis , 2016, Annals of the rheumatic diseases.

[7]  C. Wouters,et al.  2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis , 2016, Annals of the rheumatic diseases.

[8]  C. Wouters,et al.  Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis , 2016, RMD Open.

[9]  L. Luzzatto,et al.  Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry , 2016, The Journal of allergy and clinical immunology.

[10]  B. Degar.  Familial Hemophagocytic Lymphohistiocytosis. , 2015, Hematology/oncology clinics of North America.

[11]  R. Cron,et al.  Clinical features and correct diagnosis of macrophage activation syndrome , 2015, Expert review of clinical immunology.

[12]  T. Griffin,et al.  Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis , 2015, The Journal of Rheumatology.

[13]  K. Nichols,et al.  Hemophagocytic lymphohistiocytosis caused by dominant-negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion. , 2015, Blood.

[14]  J. Szustakowski,et al.  Whole‐Exome Sequencing Reveals Overlap Between Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis and Familial Hemophagocytic Lymphohistiocytosis , 2014, Arthritis & rheumatology.

[15]  C. Wouters,et al.  Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients , 2014, Arthritis & rheumatology.

[16]  N. Wulffraat,et al.  Performance of Current Guidelines for Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis , 2014, Arthritis & rheumatology.

[17]  Jia Zhang,et al.  Genetic Features of Late Onset Primary Hemophagocytic Lymphohistiocytosis in Adolescence or Adulthood , 2014, PloS one.

[18]  E. Behrens,et al.  Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis , 2014, Current opinion in rheumatology.

[19]  G. Hejblum,et al.  Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome , 2014, Arthritis & rheumatology.

[20]  R. Cron,et al.  Genetic Defects in Cytolysis in Macrophage Activation Syndrome , 2014, Current Rheumatology Reports.

[21]  G. Janka,et al.  Hemophagocytic syndromes--an update. , 2014, Blood reviews.

[22]  K. Sullivan,et al.  Brief Report: Alternative Activation of Laser‐Captured Murine Hemophagocytes , 2014, Arthritis & rheumatology.

[23]  B. Mehta,et al.  Life-threatening complications of adult-onset Still’s disease , 2014, Clinical Rheumatology.

[24]  A. Filipovich,et al.  Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment. , 2013, The Journal of pediatrics.

[25]  C. Eulenburg,et al.  Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis. , 2013, The Journal of pediatrics.

[26]  S. Ehl,et al.  Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis , 2013, British journal of haematology.

[27]  Sebastian F N Bode,et al.  Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis , 2012, Arthritis Research & Therapy.

[28]  R. Cron,et al.  Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment , 2012, Genes and Immunity.

[29]  J. Meller,et al.  Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH. , 2011, Blood.

[30]  A. Filipovich,et al.  How I treat hemophagocytic lymphohistiocytosis. , 2011, Blood.

[31]  G. Koretzky,et al.  Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice. , 2011, The Journal of clinical investigation.

[32]  N. Wulffraat,et al.  Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. , 2010, Rheumatology.

[33]  V. Ziaee,et al.  Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders , 2008, Pediatric Rheumatology Online Journal.

[34]  R. Cron,et al.  Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). , 2008, The Journal of rheumatology.

[35]  A. Ramanan,et al.  Recognition and management of macrophage activation syndrome in juvenile arthritis , 2007, Current opinion in rheumatology.

[36]  M. Aricò,et al.  HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis , 2007, Pediatric blood & cancer.

[37]  A. Martini,et al.  Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. , 2005, The Journal of pediatrics.

[38]  Frank E. Harrell,et al.  Regression Modeling Strategies: With Applications to Linear Models, Logistic Regression, and Survival Analysis , 2001 .

[39]  大杉 夕子.  Cytokine Production Regulating Th1 and Th2 Cytokines in Hemophagocytic Lymphohistiocytosis , 1998 .

[40]  E. Jaffe,et al.  Contemporary classification of histiocytic disorders , 1997 .

[41]  R. Egeler,et al.  HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. , 1997, Medical and pediatric oncology.

[42]  A. Fischer,et al.  Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. , 1996, Leukemia.

[43]  A. Prieur,et al.  Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases. , 1993, Clinical and experimental rheumatology.

[44]  U. Andersson,et al.  Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. , 1991, Blood.

[45]  G. Janka,et al.  Familial and acquired hemophagocytic lymphohistiocytosis. , 2012, Annual review of medicine.

[46]  M. Suarez‐Almazor,et al.  International League of Associations for Rheumatology: International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001 , 2004 .

[47]  J. Stéphan,et al.  [Reactive hemophagocytic syndrome in children]. , 2000, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

[48]  E. Jaffe,et al.  Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. , 1997, Medical and pediatric oncology.