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Double heterozygous sickling disorder

Last edited: 4/10/2026

Overview

Sickling disorders, including double heterozygous states, are characterized by the presence of hemoglobin S within red blood cells, which can lead to a variety of ocular complications 1. These manifestations range from benign clinical signs to severe, vision-threatening conditions such as proliferative retinopathy 1.

Diagnosis

  • High-risk lesions: Hyphema in any patient with hemoglobin S-containing red cells requires immediate identification 1.
  • Vision-threatening findings: Retinal neovascularization (proliferative retinopathy) and subsequent vitreous hemorrhage 1.
  • Benign clinical signs: The "comma sign" (conjunctival sickling), angioid streaks, and abnormal color vision are typically not associated with significant vision loss 1.

    • Management

  • Urgent Referral: Immediate ophthalmologic referral is mandatory for any patient with hemoglobin S who develops a hyphema 1.
  • Routine Referral: Patients presenting with retinal neovascularization or vitreous hemorrhage should be referred for specialized ophthalmic care 1.
  • Adjunctive Therapies: The efficacy of preoperative transfusion and experimental antisickling therapies in the prevention or treatment of ocular lesions remains incompletely defined 1.

    • Key Recommendations

  • Refer all patients with hemoglobin S and hyphema to an ophthalmologist immediately due to the high risk of complications 1. (Evidence: Expert opinion)
  • Ensure patients with proliferative retinopathy or vitreous hemorrhage receive ophthalmologic referral for management 1. (Evidence: Expert opinion)
  • Distinguish between vision-threatening lesions and benign signs (e.g., comma sign, angioid streaks) to prioritize clinical urgency 1. (Evidence: Expert opinion)

    • References

    1 Charache S. Eye disease in sickling disorders. Hematology/oncology clinics of North America 1996. link70406-8)

    Original source

    1. [1]
      Eye disease in sickling disorders. — Charache S Hematology/oncology clinics of North America (1996)
    2. [2]
      Sicklers surviving childhood in Tanzania. — Corachan M, Oomen HA, Kigadye FC, Morris H Tropical and geographical medicine (1979)
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