{
  "abstract": "Introduction: Dengue fever is a known cause of hemophagocytic lymphohistiocytosis (HLH) and can trigger autoimmune hemolytic anemia (AIHA). However, the simultaneous presentation of these conditions in pediatrics has not yet been reported. Case Description: A 4-year-old male with prior history of AIHA at 2 years old (treated with immunoglobulin and without subsequent follow-up), was admitted straight to the intensive care unit due to severe anemia and cardiac insufficiency. Laboratory tests from the referring center had shown pancytopenia (hemoglobin 3.5 g/dL, leukocytes 860mm³, platelets 136,000mm³), ferritin of 50,000µg/L, a positive direct Coombs test and a positive test for dengue virus type 1. He was initially diagnosed with Evans syndrome, most likely triggered by the adjacent infection, and treated with intravenous immunoglobulin (IVIG) and methylprednisolone. Due to persistent fever and worsening hematological parameters he underwent bone marrow examination, which revealed moderate dyserythropoiesis and hemophagocytosis, which, in conjunction with laboratory results (hemoglobin < 9 lt; 9 g/dL, neutrophils < 1,000/mm³, triglycerides ≥300 mg/dL, ferritin ≥ 500 µg/L), established the diagnosis of HLH. Dexamethasone was administered, but etoposide was not initiated at that time due to the patient’s clinical instability, with severe hypotension and acute respiratory failure. Computed tomography revealed cervical and mesenteric lymphadenopathy. Lymphoproliferative diseases were ruled out by biopsy. He required multicle packed red blood cell transfusions and received 2 more doses of IVIG, as tests continued to show a positive hemolysis profile. Once stabilized, he received 2 doses of etoposide, the first one being on the 14th day of hospitalization, and showed good hematological response, however, the third dose had to be postponed due to worsening of his clinical condition, which included fungal endocarditis by Candida tropicalis and multiple bacterial infections. The HLH treatment protocol was finally reinitiated on the 50th day of hospitalization, as he remained febrile with a declining neutrophil count despite being clinically stable. He received 6 cycles of dexamethasone (10 mg/m²/day for 3 days every 2 weeks) and 5 cycles of etoposide (150 mg/m² every 2 weeks). Given the persistence of a positive hemolysis profile and a positive direct Coombs test, cyclosporine was administered at 6 mg/m² per dose every 12 hours. Tapering of cyclosporine was initiated after 1 year of continuous use and completed 4 months later. Since then, the patient has not required any continuous medications. Currently, he remains under outpatient follow-up with Pediatric Hematology and is clinically and laboratorially stable one month after cyclosporine discontinuation. Given the history of autoimmune disease and severe infections, the patient was referred to Rheumatology, which ruled out systemic lupus erythematosus, as well as for follow-up and further evaluation with Pediatric Immunology, which is still ongoing. Conclusion: Although dengue is a well-established cause of hemophagocytic lymphohistiocytosis, the simultaneous occurrence of AIHA is extremely rare. Early recognition and prompt management are essential to improve outcomes. In this report, symptom overlap between conditions made the diagnosis particularly challenging, as the hyperinflammatory state and pancytopenia could also be attributed to sepsis and reactivation of autoimmune hemolytic anemia.",
  "abstract_shown": true,
  "affiliations": [
    "Hospital Universitário Evangélico Mackenzie, Curitiba, PR, Brazil",
    "Faculdade Evangélica Mackenzie do Paraná, Curitiba, PR, Brazil"
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 5,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Marcela QUERINO DO AMARAL"
    },
    {
      "affs": [
        0
      ],
      "name": "Tatyane BORGES CALEGARI"
    },
    {
      "affs": [
        0
      ],
      "name": "Maria Eduarda DE SOUZA DO AMARAL"
    },
    {
      "affs": [
        0
      ],
      "name": "Katerin MARTINS DEMOZZI"
    },
    {
      "affs": [
        1
      ],
      "name": "Júlia RIEDEL DERETI"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/11asv21qzrmbj.bib",
  "cited_by": 0,
  "doi": "10.1016/j.htct.2026.108364",
  "fulltext_url": "http://www.sciencedirect.com/science/article/pii/S2531137926021115",
  "id": "11asv21qzrmbj",
  "issns": [
    "2531-1379",
    "2531-1387"
  ],
  "landing_url": "https://doi.org/10.1016/j.htct.2026.108364",
  "language": "en",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "journal",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "lcc",
      "RC633-647.5"
    ],
    [
      "openalex",
      "2720|Autoimmune and Inflammatory Disorders Research"
    ]
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  "openalex": "W7223156895",
  "pdf_url": null,
  "pmcid": null,
  "pmid": null,
  "published": "2026-10-23",
  "publisher": "Elsevier",
  "ris": "https://research.draconitic.com/paper/11asv21qzrmbj.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T13:43:27Z",
      "native_id": "c157452b3ed74c869503e3550fa874ad",
      "source": "doaj",
      "url": "https://doi.org/10.1016/j.htct.2026.108364"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "lcc:RC633-647.5",
      "subfield": "endocrinology-metabolism"
    }
  ],
  "title": "HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DENGUE FEVER IN A PEDIATRIC PATIENT WITH HISTORY OF AUTOIMMUNE HEMOLYTIC ANEMIA",
  "title_text": "HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DENGUE FEVER IN A PEDIATRIC PATIENT WITH HISTORY OF AUTOIMMUNE HEMOLYTIC ANEMIA",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T13:43:27Z",
  "url": "https://research.draconitic.com/paper/11asv21qzrmbj",
  "venue": "Hematology, Transfusion and Cell Therapy",
  "versions": []
}