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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 Minoia ,
Francesca Bovis ,
Sergio Davì ,
Antonella Insalaco ,
Kai Lehmberg ,
Susan Shenoi ,
Sheila Weitzman ,
Graciela Espada ,
Yi-Jin Gao ,
Jordi Anton ,
Toshiyuki Kitoh ,
Ozgur Kasapcopur ,
Helga Sanner ,
Rosa Merino ,
Itziar Astigarraga ,
Maria Alessio ,
Michael Jeng ,
Vyacheslav Chasnyk ,
Kim E. Nichols ,
Zeng Huasong ,
Caifeng Li ,
Concetta Micalizzi ,
Nicolino Ruperto ,
Alberto Martini ,
Randy Q. Cron ,
Angelo Ravelli ,
AnnaCarin Horne ,
Mario Abinun ,
Amita Aggarwal ,
Jonathan Akikusa ,
Sulaiman Al-Mayouf ,
Maria Alessio ,
Jordi Anton ,
Maria Teresa Apaz ,
Itziar Astigarraga ,
Tadej Avcin ,
Nuray Aktay Ayaz ,
Patrizia Barone ,
Bianca Bica ,
Isabel Bolt ,
Francesca Bovis ,
Luciana Breda ,
Vyacheslav Chasnyk ,
Rolando Cimaz ,
Fabrizia Corona ,
Randy Q. Cron ,
Ruben Cuttica ,
Sergio Davì ,
Zane Davidsone ,
Carmen De Cunto ,
Jaime De Inocencio ,
Erkan Demirkaya ,
Eli M. Eisenstein ,
Sandra Enciso ,
Graciela Espada ,
Michel Fischbach ,
Michael Frosch ,
Romina Gallizzi ,
Maria Luz Gamir ,
Yi-Jin Gao ,
Thomas Griffin ,
Alexei Grom ,
Soad Hashad ,
Teresa Hennon ,
Jan-Inge Henter ,
AnnaCarin Horne ,
Gerd Horneff ,
Zeng Huasong ,
Adam Huber ,
Norman Ilowite ,
Antonella Insalaco ,
Maka Ioseliani ,
Michael Jeng ,
Agneza Marija Kapović ,
Ozgur Kasapcopur ,
Raju Khubchandani ,
Toshiyuki Kitoh ,
Isabelle Koné-Paut ,
Sheila Knupp Feitosa de Oliveira ,
Bianca Lattanzi ,
Kai Lehmberg ,
Loredana Lepore ,
Caifeng Li ,
Jeffrey M. Lipton ,
Silvia Magni-Manzoni ,
Despoina Maritsi ,
Alberto Martini ,
Deborah McCurdy ,
Rosa Merino ,
Concetta Micalizzi ,
Paivi Miettunen ,
Francesca Minoia ,
Velma Mulaosmanovic ,
Kim E. Nichols ,
Susan Nielsen ,
Seza Ozen ,
Priyankar Pal ,
Sampath Prahalad ,
Angelo Ravelli ,
Donato Rigante ,
Ingrida Rumba-Rozenfelde ,
Nicolino Ruperto ,
Ricardo Russo ,
Claudia Saad Magalhães ,
Helga Sanner ,
Wafaa Mohamed Saad Sewairi ,
Susan Shenoi ,
Clovis Artur Silva ,
Valda Stanevicha ,
Gary Sterba ,
Kimo C. Stine ,
Gordana Susic ,
Flavio Sztajnbok ,
Syuji Takei ,
Ralf Trauzeddel ,
Elena Tsitsami ,
Erbil Unsal ,
Yosef Uziel ,
Olga Vougiouka ,
Carol A. Wallace ,
Lehn Weaver ,
Jennifer E. Weiss ,
Sheila Weitzman ,
Carine Wouters ,
Nico Wulffraat ,
Mabruka Zletni ,
Maurizio Arico ,
R. Maarten Egeler ,
Alexandra H. Filipovich ,
Helmut Gadner ,
Shinsaku Imashuku ,
Gritta Janka ,
Stephan Ladisch ,
Ken L. McClain ,
David Webb
October 2017
October 2017
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Abstract
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.
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