Francesca Minoia1, Francesca Bovis2, Sergio Davì3, Antonella Insalaco4, Kai Lehmberg5, Susan Shenoi6, Sheila Weitzman7, Graciela Espada8, Yi-Jin Gao9, Jordi Anton10, Toshiyuki Kitoh11, Ozgur Kasapcopur12, Helga Sanner13, Rosa Merino14, Itziar Astigarraga15, Maria Alessio16, Michael Jeng17, Vyacheslav Chasnyk18, Kim E Nichols19, Zeng Huasong20, Caifeng Li21, Concetta Micalizzi3, Nicolino Ruperto3, Alberto Martini3, Randy Q Cron22, Angelo Ravelli23, AnnaCarin Horne24. 1. G. Gaslini Institute, Genoa, Italy. Electronic address: francescaminoia@gaslini.org. 2. University of Genova, Genoa, Italy. 3. G. Gaslini Institute, Genoa, Italy. 4. Pediatric Hospital of Bambino Gesù, Rome, Italy. 5. University Medical Center, Hamburg, Germany. 6. Seattle Children's Hospital and University of Washington, Seattle, WA. 7. The Hospital for Sick Children, Toronto, Ontario, Canada. 8. Ricardo Gutierrez Children's Hospital, Buenos Aires, Argentina. 9. Children's Hospital of Fudan University, Shanghai, China. 10. Hospital Saint Joan de Déu, Barcelona, Spain. 11. Aichi Medical University, Nagakute, Japan. 12. Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. 13. Oslo University Hospital, Rikshospitalet, Oslo, Norway. 14. La Paz University Hospital, Madrid, Spain. 15. BioCruces Health Research Institute, Cruces University Hospital, University of the Basque Country, Barakaldo, Spain. 16. University of Naples Federico II, Naples, Italy. 17. Stanford School of Medicine, Palo Alto, CA. 18. Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia. 19. St. Jude Children's Research Hospital, Memphis, TN. 20. Guangzhou Children's Hospital, Guangzhou, China. 21. Beijing Children's Hospital, Beijing, China. 22. University of Alabama, Birmingham, AL. 23. G. Gaslini Institute, Genoa, Italy; University of Genova, Genoa, Italy. 24. Karolinska University Hospital, Stockholm, Sweden.
Abstract
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 <1% for a score of <11 to >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.
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 <1% for a score of <11 to >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.
Authors: Michael B Jordan; Carl E Allen; Jay Greenberg; Michael Henry; Michelle L Hermiston; Ashish Kumar; Melissa Hines; Olive Eckstein; Stephan Ladisch; Kim E Nichols; Carlos Rodriguez-Galindo; Birte Wistinghausen; Kenneth L McClain Journal: Pediatr Blood Cancer Date: 2019-07-24 Impact factor: 3.167
Authors: Katayoun Rezvani; Sattva S Neelapu; Elizabeth J Shpall; Kris M Mahadeo; Sajad J Khazal; Hisham Abdel-Azim; Julie C Fitzgerald; Agne Taraseviciute; Catherine M Bollard; Priti Tewari; Christine Duncan; Chani Traube; David McCall; Marie E Steiner; Ira M Cheifetz; Leslie E Lehmann; Rodrigo Mejia; John M Slopis; Rajinder Bajwa; Partow Kebriaei; Paul L Martin; Jerelyn Moffet; Jennifer McArthur; Demetrios Petropoulos; Joan O'Hanlon Curry; Sarah Featherston; Jessica Foglesong; Basirat Shoberu; Alison Gulbis; Maria E Mireles; Lisa Hafemeister; Cathy Nguyen; Neena Kapoor Journal: Nat Rev Clin Oncol Date: 2019-01 Impact factor: 66.675