The conjunctival sickling sign, hemoglobin S, and irreversibly sickled erythrocytes

Area of Science:

• Ophthalmology
• Hematology
• Genetics

Background:

• Sickle cell disease encompasses several hemoglobinopathies including hemoglobins AS, SC, and SS.
• Conjunctival manifestations are common in sickle cell disease, reflecting systemic disease severity.
• Assessing conjunctival signs can provide insights into the underlying pathophysiology.

Purpose of the Study:

• To investigate the correlation between conjunctival sickling signs and vasculopathy severity with specific hematological parameters in patients with hemoglobins AS, SC, and SS.
• To determine the relationship between irreversibly sickled cells and sickle hemoglobin percentage with observed conjunctival findings.

Main Methods:

• A masked investigation was conducted on sixty-five patients diagnosed with hemoglobins AS, SC, and SS.
• Evaluated the severity of conjunctival sickling signs and conjunctival vasculopathy.
• Quantified the counts of irreversibly sickled cells and the percentage of sickle hemoglobin.

Main Results:

• A statistically significant positive correlation was found between the severity of conjunctival sickling signs and the counts of irreversibly sickled cells across all investigated hemoglobinopathy groups.
• A statistically significant positive correlation was observed between the severity of conjunctival vasculopathy and the percentage of sickle hemoglobin in the total patient cohort.

Conclusions:

• Conjunctival findings in sickle cell disease are quantitatively linked to specific hematological markers.
• The severity of conjunctival sickling and vasculopathy can serve as indicators of disease burden related to irreversibly sickled cells and sickle hemoglobin levels.