https://doi.org/10.4081/reumatismo.2022.1488
Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage
Submitted: 12 March 2022
Accepted: 1 January 2023
Published: 21 March 2023
Accepted: 1 January 2023
Abstract Views: 1053
PDF: 524
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by macrophages and cytotoxic T cells with aberrant activation. The primary (genetic) form, which is caused by mutations that affect lymphocyte cytotoxicity and immune regulation, is most prevalent in children, whereas the secondary (acquired) form is prevalent in adults. Secondary HLH is commonly caused by infections or cancers, but it can also be caused by autoimmune disorders, in which case it is known as macrophage activation syndrome (MAS; or MAS-HLH). A 25-year-old female presented with a high-grade fever that lasted for two weeks. His laboratory results revealed pancytopenia, neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Based on the clinical presentation and laboratory findings, a provisional diagnosis of HLH has been made. A HLH protocol was utilized to treat the patient. During the course of hospitalization, systemic lupus erythematosus (SLE) was identified as the underlying cause. She improved dramatically after receiving an immunosuppressive regimen of etoposide, cyclosporine, and dexamethasone according to HLH protocol-2004 with individualized modifications. The clinician should be aware that HLH may be the initial manifestation of underlying SLE. Early diagnosis and aggressive, individualized treatment are the key to improving outcomes.
Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010; 16: S82-89.
DOI: https://doi.org/10.1016/j.bbmt.2009.11.014
Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, et al. Pediatric Rheumatology International Trials Organization; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014; 66: 3160- 3169.
DOI: https://doi.org/10.1002/art.38802
Borgia RE, Gerstein M, Levy DM, Silverman ED, Hiraki LT. Features, treatment, and outcomes of macrophage activation syndrome in childhood-onset systemic lupus erythematosus. Arthritis Rheumatol. 2018; 70: 616-624.
DOI: https://doi.org/10.1002/art.40417
Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48: 124-131.
DOI: https://doi.org/10.1002/pbc.21039
Imashuku S, Hlbi S, Todo S. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr. 1997; 130: 352-357.
DOI: https://doi.org/10.1016/S0022-3476(97)70195-1
Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008; 50: 1227-1235.
DOI: https://doi.org/10.1002/pbc.21423
Zhou J, Zhou J, Shen DT, Goyal H, Wu ZQ, Xu HG. Development and validation of the prognostic value of ferritin in adult patients with Hemophagocytic Lymphohistiocytosis. Orphanet J Rare Dis. 2020; 15: 71.
DOI: https://doi.org/10.1186/s13023-020-1336-6
Song Y, Pei RJ, Wang YN, Zhang J, Wang Z. Central nervous system involvement in hemophagocytic lymphohistiocytosis in adults: a retrospective analysis of 96 patients in a single center. Chin Med J (Engl). 2018; 131: 776-783.
DOI: https://doi.org/10.4103/0366-6999.228234
Horne A, Wickström R, Jordan MB, Yeh EA, Naqvi A, Henter JI, et al. How to treat involvement of the central nervous system in hemophagocytic lymphohistiocytosis? Curr Treat Options Neurol. 2017; 19:3.
DOI: https://doi.org/10.1007/s11940-017-0439-4
Kim MM, Yum MS, Choi HW, Ko TS, Im HJ, Seo JJ, et al. Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis. Korean J Hematol. 2012; 47: 273-280.
DOI: https://doi.org/10.5045/kjh.2012.47.4.273
Rivière S, Galicier L, Coppo P, Marzac C, Au- mont C, Lambotte O, et al. Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients. Am J Med. 2014; 1 27: 1118-1125.
DOI: https://doi.org/10.1016/j.amjmed.2014.04.034
Bartynski WS. Posterior reversible encepha- lopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol. 2008; 29: 1043-1049.
DOI: https://doi.org/10.3174/ajnr.A0929
How to Cite
Sukhadiya, P., Kumar, P., Meena, D., Kumar P.H., A., Vijayan, N., Garg, P., & Garg, M. (2023). Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage. Reumatismo, 74(4). https://doi.org/10.4081/reumatismo.2022.1488
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