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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Inflammation in sickle cell disease.

Nicola Conran1, John D Belcher2

  • 1Hematology Center, University of Campinas - UNICAMP, Cidade Universitária, Campinas-SP, Brazil.

Clinical Hemorheology and Microcirculation
|April 5, 2018
PubMed
Summary
This summary is machine-generated.

Sickle cell disease (SCD) involves inflammation triggering vaso-occlusion and complications. Targeting these inflammatory pathways offers new therapeutic strategies for SCD management.

Keywords:
Cytokineendotheliumhemolysishydroxyureavaso-occlusion

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Area of Science:

  • Hematology
  • Immunology
  • Pathophysiology

Background:

  • Sickle cell disease (SCD) is caused by a primary β-globin gene mutation.
  • This mutation leads to hemolysis, inflammation, and vaso-occlusion.
  • Inflammation is central to SCD pathophysiology and its complications.

Purpose of the Study:

  • To discuss triggers, mechanisms, molecules, and cells involved in SCD inflammation.
  • To highlight the role of inflammation in SCD-related complications.
  • To explore anti-inflammatory therapies as potential SCD treatments.

Main Methods:

  • Review of existing literature on SCD pathophysiology and inflammation.
  • Discussion of inflammatory pathways and cellular players in SCD.
  • Analysis of current and developing therapeutic strategies targeting inflammation.

Main Results:

  • Inflammatory processes are initiated by the primary β-globin gene mutation.
  • Inflammation drives acute vaso-occlusive episodes and chronic complications.
  • Numerous inflammatory molecules and cells propagate these processes.

Conclusions:

  • Inflammation is a critical therapeutic target in sickle cell disease.
  • Anti-inflammatory drugs show promise for SCD treatment, potentially alongside or replacing hydroxyurea.
  • Targeting inflammation may offer new options for patients ineligible for stem cell or gene therapies.