{
  "abstract": "Introduction: Sickle cell disease (SCD) remains associated with substantial morbidity and premature mortality despite major advances in disease-modifying therapies. However, contemporary multicenter data describing the clinical characteristics, disease burden, and treatment patterns of individuals with SCD in Brazil remain scarce. Objectives: We aimed to characterize the clinical and treatment profile of participants enrolled in the Recipient Epidemiology and Donor Evaluation Study-IV-Pediatric (REDS-IV-P) Brazil SCD cohort. Methods: REDS-IV-P is a prospective multicenter cohort conducted at eight Brazilian referral centers: HCFMUSP and ITACI (São Paulo), Fundação Hemominas (Belo Horizonte, Juiz de Fora, and Montes Claros), Hemope (Recife), Hemorio (Rio de Janeiro), and Hemoam (Manaus). Demographic characteristics, laboratory parameters, treatment utilization, SCD-related complications, and hospitalizations were collected using standardized procedures and uniform, protocol-defined outcome definitions across all participating centers. SCD-related outcomes were evaluated according to genotype (HbSS, HbSβ⁰, and HbSD-Punjab grouped as sickle cell anemia [SCA]) and age group (<18 vs. ≥18-years). Results: Of the 3,049 participants, there was a slight predominance of adults (55.6%) and females (54.4%). HbSS was the most common genotype (72.4%), followed by HbSC (21.6%), HbSβ⁰ (2.9%), and HbSβ+ (2.8%). Participants with SCA had significantly higher white blood cell and platelet counts, bilirubin and lactate dehydrogenase levels, and lower hemoglobin concentrations than other genotypes. Hydroxyurea was used by 57.5% of the overall cohort and by 70.0% of participants with SCA, whereas chronic transfusion therapy was used by approximately one-fifth of individuals with SCA. Among adults, avascular necrosis affected 24.9%, 22.7%, and 33.3% of participants with SCA, HbSC, and HbSβ+, respectively, while ischemic stroke was observed in 18.4%, 3.5%, and 9.5%, and leg ulcers in 17.6%, 3.5%, and 19.1%. Splenic sequestration affected 40.0% of children with SCA. During follow-up, SCA showed the highest incidence rates of ischemic stroke (0.18 vs. 0.07 and 0.00 events per 100 person-yrs), bacteremia/sepsis (0.45 vs. 0.00 and 0.00), and leg ulcers (0.85 vs. 0.14 and 0.00) compared with HbSC and HbSβ+, respectively. Hospitalization rates for acute chest syndrome and vaso-occlusive episodes were consistently highest among participants with SCA, whereas all-cause hospitalization rates among adults were highest in HbSβ+ disease. Hydroxyurea utilization varied substantially across participating centers, with the lowest use observed in Montes Claros and the highest in HEMOAM. Discussion: Although access to hydroxyurea and chronic transfusion therapy has expanded substantially in Brazil over the past decade, the persistence of frequent acute complications, chronic organ damage, and hospitalizations, together with persistent regional disparities in treatment implementation, demonstrates that current therapeutic strategies remain insufficient to fully prevent disease progression. Conclusions: The REDS-IV-P SCD cohort demonstrates that despite broad use of disease-modifying therapies, individuals with SCD in Brazil continue to experience a considerable burden of acute and chronic complications. These findings provide an updated national overview of SCD and support continued efforts to optimize treatment strategies and expand access to effective and curative therapies.",
  "abstract_shown": true,
  "affiliations": [
    "Fundação Hemominas (FH), Belo Horizonte, MG, Brazil",
    "Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, SP, Brazil",
    "Westat, United States",
    "Universidade de São Paulo (USP), São Paulo, SP, Brazil",
    "Universidade São Caetano do Sul (USCS), São Caetano do Sul, SP, Brazil",
    "Fundação Hemominas (FH), Montes Claros, MG, Brazil",
    "Fundação Hemominas, Juiz de Fora, MG, Brazil",
    "Fundação de Hematologia e Hemoterapia de Pernambuco (Hemope), Recife, PE, Brazil",
    "Hemorio, Rio de Janeiro, RJ, Brazil",
    "Fundação HEMOAM, Manaus, AM, Brazil",
    "Johns Hopkins All Children's Hospital, United States",
    "University of British Columbia, United States",
    "Vitalant Research Institute, United States",
    "UCSF Benioff Children’s Hospital Oakland, United States"
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 21,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "André Rolim BELISÁRIO"
    },
    {
      "affs": [
        1
      ],
      "name": "Carla Luana DINARDO"
    },
    {
      "affs": [
        2
      ],
      "name": "Donald E. WARDEN"
    },
    {
      "affs": [
        2
      ],
      "name": "Sarah WILSON"
    },
    {
      "affs": [
        3
      ],
      "name": "Mina Cintho OZAHATA"
    },
    {
      "affs": [
        4
      ],
      "name": "Sheila De Oliveira Garcia MATEOS"
    },
    {
      "affs": [
        0
      ],
      "name": "Carolina Teixeira MIRANDA"
    },
    {
      "affs": [
        0
      ],
      "name": "Anna Bárbara CARNEIRO-PROIETTI"
    },
    {
      "affs": [
        5
      ],
      "name": "Rosimere Afonso MOTA"
    },
    {
      "affs": [
        6
      ],
      "name": "Daniela De Oliveira Werneck RODRIGUES"
    },
    {
      "affs": [
        3
      ],
      "name": "Ester Cerdeira SABINO"
    },
    {
      "affs": [
        3
      ],
      "name": "Ligia CAPUANI"
    },
    {
      "affs": [
        7
      ],
      "name": "Alessandra FERRAZ"
    },
    {
      "affs": [
        8
      ],
      "name": "Cláudia MÁXIMO"
    },
    {
      "affs": [
        3
      ],
      "name": "Miriam V. FLOR-PARK"
    },
    {
      "affs": [
        9
      ],
      "name": "Allyson Guimarães da COSTA"
    },
    {
      "affs": [
        3
      ],
      "name": "Vanderson ROCHA"
    },
    {
      "affs": [
        10
      ],
      "name": "Cassandra JOSEPHSON"
    },
    {
      "affs": [
        11
      ],
      "name": "Steve KLEINMAN"
    },
    {
      "affs": [
        12
      ],
      "name": "Brian CUSTER"
    },
    {
      "affs": [
        13
      ],
      "name": "Shannon KELLY"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/2z9wy8rzn4054.bib",
  "cited_by": 0,
  "doi": "10.1016/j.htct.2026.106562",
  "fulltext_url": "http://www.sciencedirect.com/science/article/pii/S2531137926003093",
  "id": "2z9wy8rzn4054",
  "issns": [
    "2531-1379",
    "2531-1387"
  ],
  "landing_url": "https://doi.org/10.1016/j.htct.2026.106562",
  "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",
      "2716|Hemoglobinopathies and Related Disorders"
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  "openalex": "W7223578164",
  "pdf_url": null,
  "pmcid": null,
  "pmid": null,
  "published": "2026-10-23",
  "publisher": "Elsevier",
  "ris": "https://research.draconitic.com/paper/2z9wy8rzn4054.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:17:29Z",
      "native_id": "1d7175e224f2416dbdaeb46a0f88c400",
      "source": "doaj",
      "url": "https://doi.org/10.1016/j.htct.2026.106562"
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  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2716|Hemoglobinopathies and Related Disorders",
      "subfield": "human-genetics"
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  ],
  "title": "CONTEMPORARY SICKLE CELL DISEASE IN BRAZIL: FINDINGS FROM A MULTICENTER COHORT OF 3,049 PARTICIPANTS",
  "title_text": "CONTEMPORARY SICKLE CELL DISEASE IN BRAZIL: FINDINGS FROM A MULTICENTER COHORT OF 3,049 PARTICIPANTS",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T13:43:18Z",
  "url": "https://research.draconitic.com/paper/2z9wy8rzn4054",
  "venue": "Hematology, Transfusion and Cell Therapy",
  "versions": []
}