HLH Syndrome in a Community Hospital: The Challenge of an Early Diagnosis

References

• Hosahalli Vasanna S, Dalal J. Traffic jam within lymphocytes: a clinician’s perspective. Front Immunol. 2022;13:1034317. doi:10.3389/fimmu.2022.1034317
   PubMed Web of Science ®Google Scholar
• Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020;135(16):1332–1343. doi:10.1182/blood.2019000936
   PubMed Web of Science ®Google Scholar
• Galletta F, Cucinotta U, Marseglia L, et al. Hemophagocytic lymphohistiocytosis following gene replacement therapy in a child with type 1 spinal muscular atrophy. J Clin Pharm Ther. 2022;47(9):1478–1481. doi:10.1111/jcpt.13733
   PubMed Web of Science ®Google Scholar
• Niece JA, Rogers ZR, Ahmad N, Langevin AM, McClain KL. Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer. 2010;54(3):424–428. doi:10.1002/pbc.22359
   PubMed Web of Science ®Google Scholar
• Henter JI, Elinder G, Söder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Padiatric Scand. 1991;80(4):428–435. doi:10.1111/j.1651-2227.1991.tb11878.x
   PubMed Web of Science ®Google Scholar
• Jordan MB, Allen CE, Greenberg J, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019;66(11):e27929. doi:10.1002/pbc.27929
   PubMed Web of Science ®Google Scholar
• Minoia F, Bovis F, Davì S, et al. Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis. 2019;78(10):1357–1362. doi:10.1136/annrheumdis-2019-215211
   PubMed Web of Science ®Google Scholar
• Ravelli A, Minoia F, Davì S, et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: a European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2016;68(3):566–576. doi:10.1002/art.39332
   PubMed Web of Science ®Google Scholar
• Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613–2620. doi:10.1002/art.38690
   PubMed Web of Science ®Google Scholar
• Henter JI, Horne A, Aricó M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–131. doi:10.1002/pbc.21039
   PubMed Web of Science ®Google Scholar
• Rubin TS, Zhang K, Gifford C, et al. Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH. Blood. 2017;129(22):2993–2999. doi:10.1182/blood-2016-12-753830
   PubMed Web of Science ®Google Scholar
• Chaturvedi V, Marsh RA, Zoref-Lorenz A, et al. T-cell activation profiles distinguish hemophagocytic lymphohistiocytosis and early sepsis. Blood. 2021;137(17):2337–2346. doi:10.1182/blood.2020009499
   PubMed Web of Science ®Google Scholar
• Amirifar P, Ranjouri MR, Abolhassani H, et al. Clinical, immunological and genetic findings in patients with UNC13D deficiency (FHL3): a systematic review. Pediatr Allergy Immunol. 2021;32(1):186–197. doi:10.1111/pai.13323
   PubMed Web of Science ®Google Scholar
• Otto WR, Behrens EM, Teachey DT, et al. Human Adenovirus 7-Associated Hemophagocytic Lymphohistiocytosis-like Illness: clinical and Virological Characteristics in a Cluster of Five Pediatric Cases. Clin Infect Dis. 2021;73(7):e1532–e1538. doi:10.1093/cid/ciaa1277
   PubMed Web of Science ®Google Scholar
• La Fay C, Bosdure E, Baravalle-Einaudi M, Stremler-Le Bel N, Dubus JC, Mazenq J. Severe adenovirus pneumonia with hemophagocytic syndrome and respiratory failure. Arch Pediatr. 2020;27(7):383–385. doi:10.1016/j.arcped.2020.07.003
   PubMed Web of Science ®Google Scholar
• Zhang HY, Li CJ, Long Y, Sun DM, Wang RG, Zhang Y. Clinical features of children with severe adenovirus pneumonia and hemophagocytic syndrome: an analysis of 30 cases. Zhongguo Dang Dai Er Ke Za Zhi. 2020;22(7):744–748. doi:10.7499/j.issn.1008-8830.2003080
   PubMedGoogle Scholar
• Singh P, Secord E, Pappas K, Savaşan S. An infant with severe combined immunodeficiency, osteopetrosis, chromosomally integrated herpesvirus-6 infection, and hemophagocytic syndrome: what are the links? Pediatr Blood Cancer. 2021;68(1):e28564. doi:10.1002/pbc.28564
   PubMed Web of Science ®Google Scholar
• Inoue K, Miura H, Hoshino A, et al. Inherited chromosomally integrated human herpesvirus-6 in a patient with XIAP deficiency. Transpl Infect Dis. 2020;22(5):e13331. doi:10.1111/tid.13331
   PubMed Web of Science ®Google Scholar
• Bucciol G, Willemyns N, Verhaaren B, et al. Child Neurology: familial Hemophagocytic Lymphohistiocytosis Underlying Isolated Central Nervous System Inflammation. Neurology. 2022;99(15):660–664. doi:10.1212/WNL.0000000000201124
   PubMed Web of Science ®Google Scholar
• Choi YB, Yi DY. Fatal case of hemophagocytic lymphohistiocytosis associated with group B streptococcus sepsis: a case report. Medicine. 2018;97(40):e12210. doi:10.1097/MD.0000000000012210
   PubMed Web of Science ®Google Scholar
• Marsh RA. Epstein-Barr Virus and Hemophagocytic Lymphohistiocytosis. Front Immunol. 2017;8:1902. doi:10.3389/fimmu.2017.01902
   PubMed Web of Science ®Google Scholar
• López-Nevado M, Sevilla J, Almendro-Vázquez P, et al. Inborn Error of STAT2-Dependent IFN-I Immunity in a Patient Presented with Hemophagocytic Lymphohistiocytosis and Multisystem Inflammatory Syndrome in Children. J Clin Immunol. 2023;43(6):1278–1288. doi:10.1007/s10875-023-01488-6
   PubMed Web of Science ®Google Scholar
• Wang W, Gong F, Zhu W, Fu S, Zhang Q. Macrophage activation syndrome in Kawasaki disease: more common than we thought? Semin Arthritis Rheum. 2015;44(4):405–410. doi:10.1016/j.semarthrit.2014.07.007
   PubMed Web of Science ®Google Scholar