A need for greater awareness: a mixed methods study of patient and healthcare professional perspectives on the diagnosis journey for haemophagocytic lymphohistiocytosis (HLH)

References

• Allen CE, McClain KL. Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematology Am Soc Hematol Educ Program. 2015;2015:177–182. doi:10.1182/asheducation-2015.1.177
   PubMed Web of Science ®Google Scholar
• Jordan MB, Allen CE, Weitzman S, et al. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011 Oct 13;118(15):4041–4052. doi:10.1182/blood-2011-03-278127
   PubMed Web of Science ®Google Scholar
• Soy M, Atagunduz P, Atagunduz I, et al. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatol Int. 2021 Jan;41(1):7–18. doi:10.1007/s00296-020-04636-y
   PubMed Web of Science ®Google Scholar
• George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69–86. doi:10.2147/JBM.S46255
   PubMedGoogle Scholar
• Jumic S, Nand S. Hemophagocytic lymphohistiocytosis in adults: associated diagnoses and outcomes, a ten-year experience at a single institution. J Hematol. 2019 Dec;8(4):149–154. doi:10.14740/jh592
   PubMedGoogle Scholar
• Kalmuk J, Puchalla J, Feng G, et al. Pembrolizumab-induced Hemophagocytic Lymphohistiocytosis: an immunotherapeutic challenge. Cancers Head Neck. 2020;5:3. doi:10.1186/s41199-020-0050-3
   PubMedGoogle Scholar
• Grom AA, Horne A, De Benedetti F. Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol. 2016 May;12(5):259–268. doi:10.1038/nrrheum.2015.179
   PubMed Web of Science ®Google Scholar
• Crayne CB, Albeituni S, Nichols KE, et al. The immunology of macrophage activation syndrome. Front Immunol. 2019;10:119. doi:10.3389/fimmu.2019.00119
   PubMed Web of Science ®Google Scholar
• Henter JI, Horne A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124–131. doi:10.1002/pbc.21039
   PubMed Web of Science ®Google Scholar
• Henter JI, Samuelsson-Horne A, Arico M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002 Oct 1;100(7):2367–2373. doi:10.1182/blood-2002-01-0172
   PubMed Web of Science ®Google Scholar
• Ehl S, Astigarraga I, von Bahr Greenwood T, et al. Recommendations for the use of Etoposide-based therapy and bone marrow transplantation for the treatment of HLH: consensus statements by the HLH steering committee of the histiocyte society. J Allergy Clin Immunol Pract. 2018 Sep - Oct;6(5):1508–1517. doi:10.1016/j.jaip.2018.05.031
   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 Nov;66(11):e27929. doi:10.1002/pbc.27929
   PubMed Web of Science ®Google Scholar
• La Rosée P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465–2477. doi:10.1182/blood.2018894618
   PubMed Web of Science ®Google Scholar
• Bergsten E, Horne A, Arico M, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017 Dec 21;130(25):2728–2738. doi:10.1182/blood-2017-06-788349
   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 Sep;66(9):2613–2620. doi:10.1002/art.38690
   PubMed Web of Science ®Google Scholar
• Ravelli A, Minoia F, Davi S, et al. 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 Mar;68(3):566–576. doi:10.1002/art.39332
   PubMed Web of Science ®Google Scholar
• Creswell JW, Klassen AC, Plano Clark VL, et al. Best practices for mixed methods research in the health sciences. National Institutes of Health, 2011.
   Google Scholar
• Nixon A, Roddick E, Moore K, et al. A qualitative investigation into the impact of hemophagocytic lymphohistiocytosis on children and their caregivers. Orphanet J Rare Dis. 2021 May 6;16(1):205. doi:10.1186/s13023-021-01832-2
   PubMed Web of Science ®Google Scholar
• Morrissette K, Bridwell R, Lentz S, et al. Hemophagocytic lymphohistiocytosis in the emergency department: recognizing and evaluating a hidden threat. J Emerg Med. 2021 Jun;60(6):743–751. doi:10.1016/j.jemermed.2021.02.006
   PubMed Web of Science ®Google Scholar
• Ely JW, Graber ML, Croskerry P. Checklists to reduce diagnostic errors. Acad Med. 2011 Mar;86(3):307–313. doi:10.1097/ACM.0b013e31820824cd
   PubMed Web of Science ®Google Scholar
• Birndt S, Schenk T, Heinevetter B, et al. Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry. J Cancer Res Clin Oncol. 2020 Apr;146(4):1065–1077. doi:10.1007/s00432-020-03139-4
   PubMed Web of Science ®Google Scholar
• Boom V, Anton J, Lahdenne P, et al. Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Pediatric Rheumatology. 2015;13(1):55. doi:10.1186/s12969-015-0055-3
   PubMed Web of Science ®Google Scholar
• Abdirakhmanova ASV, Mukusheva Z, Assylbekova M, et al. Macrophage activation syndrome in pediatric systemic lupus erythematosus: a systematic review of the diagnostic aspects. Front Med. 2021;8:681875. doi:10.3389/fmed.2021.681875
   Web of Science ®Google Scholar
• Smits BM, van Montfrans J, Merrill SA, et al. A minimal parameter set facilitating early decision-making in the diagnosis of hemophagocytic lymphohistiocytosis. J Clin Immunol. 2021 Aug;41(6):1219–1228. doi:10.1007/s10875-021-01005-7
   PubMed Web of Science ®Google Scholar
• Merrill SA, Naik R, Streiff MB, et al. A prospective quality improvement initiative in adult hemophagocytic lymphohistiocytosis to improve testing and a framework to facilitate trigger identification and mitigate hemorrhage from retrospective analysis. Medicine (Baltimore). 2018 Aug;97(31):e11579. doi:10.1097/MD.0000000000011579
   PubMed Web of Science ®Google Scholar
• Gars E, Purington N, Scott G, et al. Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis. Haematologica. 2018 Oct;103(10):1635–1641. doi:10.3324/haematol.2017.186627
   PubMed Web of Science ®Google Scholar
• Ho C, Yao X, Tian L, et al. Marrow assessment for hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol. 2014 Jan;141(1):62–71. doi:10.1309/AJCPMD5TJEFOOVBW
   PubMed Web of Science ®Google Scholar
• Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2015 May 7;125(19):2908–2914. doi:10.1182/blood-2015-01-551622
   PubMed Web of Science ®Google Scholar
• Vanhelleputte P, Nijs K, Delforge M, et al. Pain during bone marrow aspiration: prevalence and prevention. J Pain Symptom Manage. 2003 Sep;26(3):860–866. doi:10.1016/S0885-3924(03)00312-9
   PubMed Web of Science ®Google Scholar
• Zarnegar-Lumley S, Lange KR, Mathias MD, et al. Local anesthesia with general anesthesia for pediatric bone marrow procedures. Pediatrics. 2019;144(2):e20183829. doi:10.1542/peds.2018-3829
   PubMed Web of Science ®Google Scholar