Pediatric Infectious Disease

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VOLUME 5 , ISSUE 4 ( October-December, 2023 ) > List of Articles

CASE REPORT

Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication

Ashwani K Sood, Saguna Pandit, Nidhi Chadha, Naresh I Jindal

Keywords : Case report, Cytokine storm, Dengue fever, Dengue fever with warning signs, Hemophagocytic lymphohistiocytosis

Citation Information : Sood AK, Pandit S, Chadha N, Jindal NI. Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication. Pediatr Inf Dis 2023; 5 (4):135-137.

DOI: 10.5005/jp-journals-10081-1412

License: CC BY-NC 4.0

Published Online: 14-12-2023

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a devastating and rare multisystemic disorder characterized by an autoimmune phenomenon leading to reactive hyperactivity of cytotoxic T cells and histiocytes mediated by cytokine storm. HLH could be primary (hereditary) or secondary (acquired). Unremittent fever, organomegaly, lymphadenopathy, and neurologic dysfunction are among the common manifestations of HLH, along with abnormal lab parameters like hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and transaminitis. We report a case of a 16-year-old adolescent girl presenting to the emergency department with a history of high-grade fever, with partial response to antipyretics, body aches, and severe headache for 4 days. The physical examination revealed significant hepatomegaly. enzyme-linked immunosorbent assay was positive for dengue nonstructural protein 1 (NS1) antigen (Ag) assay. The patient was managed as per the World Health Organization (WHO) protocol and started improving from dengue; however, during the second week of the illness, the patient continued to have persistent fever. A repeat lab workup revealed bicytopenia with elevated ferritin levels (17891 ng/mL). Diagnosing dengue-associated HLH is challenging unless the treating team is aware of this association, as early recognition and timely institution of immunosuppressive and specific therapy for the underlying infection is associated with improved outcomes. Physicians should collaborate with pathologists and microbiologists for early diagnosis.


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  1. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007;166(2):95–109. DOI: 10.1007/s00431-006-0258-1
  2. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet 2014;383(9927):1503–1516. DOI: 10.1016/S0140-6736(13)61048-X
  3. Munshi A, Alsuraihi A, Balubaid M, et al. Dengue-induced hemophagocytic lymphohistiocytosis: a case report and literature review. Cureus 2021;13(12):e20172. DOI: 10.7759/cureus.20172
  4. Horne A, Ramme KG, Rudd E, et al. Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis. Br J Haematol 2008;143(1): 75–83. DOI: 10.1111/j.1365-2141.2008.07315.x
  5. Ray U, Dutta S, Mondal S, et al. Severe dengue due to secondary hemophagocytic lymphohistiocytosis: a case study. Infect Dis Cases 2017;8:50–53. DOI: 10.1016/j.idcr.2017.03.013
  6. Grzybowski B, Vishwanath VA. Hemophagocytic lymphohistiocytosis: a diagnostic conundrum. J Pediatr Neurosci 2017;12(1):55–60. DOI: 10.4103/jpn.JPN_140_16
  7. Ellis EM, Sharp TM, Pérez-Padilla J, et al. Incidence and risk factors for developing dengue-associated hemophagocytic lymphohistiocytosis in Puerto Rico, 2008-2013. PLoS Negl Trop Dis 2016;10(8):e0004939. DOI: 10.1371/journal.pntd.0004939
  8. Martina BE, Koraka P, Osterhaus AD. Dengue virus pathogenesis: an integrated view. Clin Microbiol Rev 2009;22(4):564–581. DOI: 10.1128/CMR.00035-09
  9. Pradeep C, Karunathilake P, Abeyagunawardena S, et al. Hemophagocytic lymphohistiocytosis as a rare complication of dengue haemorrhagic fever: a case report. J Med Case Rep 2023;17(1):224. DOI: 10.1186/s13256-023-03967-1
  10. Chang CY, Rajappan M, Zaid M, et al. Dengue fever complicated by hemophagocytic lymphohistiocytosis: report of 2 cases and bone marrow findings. Clin Case Rep 2020;8(12):3427–3431. DOI: 10.1002/ccr3.3422
  11. Henter JI, Horne A, Arico 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
  12. La Rosée P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 2019;133(23):2465–2477. DOI: 10.1182/blood.2018894618
  13. Eloseily EM, Weiser P, Crayne CB, et al. Benefit of anakinra in treating pediatric secondary hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 2020;72(2):326–334. DOI: 10.1002/art.41103
  14. Bergsten E, Horne A, Aricó M, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood 2017;130(25):2728–2738. DOI: 10.1182/blood-2017-06-788349
  15. Henter JI, Samuelsson-Horne A, Arico M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002;100(7):2367–2373. DOI: 10.1182/blood-2002-01-0172
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