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Updated: Jun 27, 2025

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Sickle Cell Disease in Brazil: Current Management.

Aderson da Silva Araújo1, Ana Cristina Silva Pinto2, Clarisse Lopes de Castro Lobo3

  • 1Department of Hematology, Fundação de Hematologia e Hemoterapia de Pernambuco, Recife, Brazil.

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|April 25, 2024
PubMed
Summary

Sickle cell disease (SCD) is an inherited blood disorder causing red blood cell sickling and vaso-occlusion. New treatments like crizanlizumab offer hope, particularly in regions like Brazil.

Keywords:
Sickle cell diseasehemolysishydroxyureared blood cellvaso-occlusive crisis

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Area of Science:

  • Hematology
  • Genetics
  • Pharmacology

Background:

  • Sickle cell disease (SCD) is an inherited red blood cell disorder caused by a specific mutation in the β-globin gene.
  • It leads to abnormal hemoglobin S polymerization under deoxygenated conditions, resulting in red blood cell sickling, hemolysis, and vaso-occlusion.
  • Vaso-occlusive crisis (VOC) is a primary complication, but SCD can lead to other severe health issues.

Purpose of the Study:

  • To review recent data on sickle cell disease management and treatment options.
  • To focus on available therapies, particularly in the context of Brazil.
  • To highlight emerging treatments for SCD patients.

Main Methods:

  • Literature review of recent data on sickle cell disease.
  • Analysis of treatment options and their regional availability.
  • Focus on specific new therapies and their approval status.

Main Results:

  • Sickle cell disease is a growing global health concern, with increased incidence in the Americas and Africa.
  • Disease management strategies differ significantly based on regional resources and socioeconomic factors.
  • Crizanlizumab is an approved treatment option in Brazil, indicating advancements in SCD therapy.

Conclusions:

  • There is a need for updated data on sickle cell disease treatments globally.
  • Advancements in therapeutic options, such as crizanlizumab, are crucial for improving patient outcomes.
  • Ensuring equitable access to new treatments for all SCD patients is a key challenge.