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[Maculopathy in sickle cell disease].

Isabel Bachmeier1, Christiane Blecha2, Jürgen Föll3

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Summary

Sickle cell retinopathy (SCR) and sickle cell maculopathy (SCM) are ocular complications of sickle cell disease (SCD). SCM can occur early and independently of SCR, necessitating awareness for timely diagnosis.

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Area of Science:

  • Ophthalmology
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is an inherited blood disorder causing vaso-occlusive crises and organ damage.
  • Sickle cell retinopathy (SCR) is a known ocular complication, but sickle cell maculopathy (SCM) can also occur.
  • SCM may present early in SCD, independent of peripheral retinopathy.

Purpose of the Study:

  • To review ocular manifestations of SCD, focusing on SCR and SCM.
  • To discuss current systemic therapies for SCD.
  • To highlight the increasing recognition of SCM in clinical practice.

Main Methods:

  • Literature review of international and German studies on ocular involvement in SCD.
  • Focus on SCR and SCM, including diagnostic advancements.
  • Overview of systemic treatment strategies for SCD.

Main Results:

  • SCM, characterized by temporal inner retinal thinning, has gained recognition recently with advanced imaging (SD-OCT, OCTA).
  • Approximately 50% of SCD patients may develop SCM early, irrespective of SCR.
  • Increased prevalence of SCM is expected in Germany due to improved therapies and migration.

Conclusions:

  • SCM is an important, often early, ocular complication of SCD that can occur without peripheral SCR.
  • Awareness of SCM is crucial for early diagnosis and avoiding unnecessary investigations.
  • Advances in imaging and systemic treatments necessitate increased clinical vigilance for SCM in SCD patients.