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Retinopathy in haemoglobin C trait

M Hingorani1, C R Bentley, H Jackson

  • 1Moorfields Eye Hospital, London, UK.

Eye (London, England)
|January 1, 1996
PubMed
Summary
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Hemoglobin C trait, usually benign, can cause sight-threatening retinopathy and vascular occlusion, similar to sickle cell disease. This highlights the need for vigilance in patients with hemoglobin C trait, especially with coexisting conditions.

Area of Science:

  • Ophthalmology
  • Hematology
  • Genetics

Background:

  • Sickle cell disease and sickle cell trait are known causes of retinopathy.
  • Hemoglobin C trait is generally considered benign with rare systemic manifestations.
  • Hemoglobin C affects red blood cell plasticity and increases blood viscosity without causing sickling.

Observation:

  • Three cases of retinopathy in patients with hemoglobin C trait are presented.
  • Patients exhibited peripheral vascular occlusion and sea-fan formation, confirmed by fluorescein angiography.
  • Two patients had coexisting conditions: hypertension and diabetes mellitus.

Findings:

  • Hemoglobin C trait was associated with significant peripheral vascular occlusion and sea-fan neovascularization.
  • Vitreous hemorrhage was the presenting symptom in two of the three patients.

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  • These findings indicate that hemoglobin C trait can lead to sight-threatening complications.

    • Implications:

    • Hemoglobin C trait should be recognized as a potential cause of severe retinopathy.
    • Ophthalmologists should consider hemoglobin C trait in the differential diagnosis of sickle-like retinopathy.
    • Further research is needed to understand the mechanisms and risk factors for retinopathy in hemoglobin C trait.