Proliferative sickle cell retinopathy associated with sickle cell trait and gestational diabetes: case report

Sickle Cell Trait: A Benign State?

“…Though none of the cases had the required HbS concentration of >50% expected in Sβthal, it should also be noted that they also had HbA levels below 60%. In 2 of the cases [6,36] , the total of the hemoglobin fractions falls short of the expected 100%, while in the third patient, the total is slightly above 100%, which may suggest the presence of other hemoglobin fractions, rounding errors or inaccurate quantitation. The method used for hemoglobin electrophoresis was stated only in 1 case [6] and it was by high performance liquid chromatography, which may be the reason why the level of HbF was also recorded.…”

“…In addition, the data showed a higher prevalence of 29% compared to the prevalence of 8% in the African American population [16] . Though sickle cell retinopathy was also reported in a patient with gestational diabetes and hypertension [36] , this was contradicted by a study concluding that there is no association between SCT and diabetic retinopathy after comparing diabetic patients with and without retinopathy. This again is despite the fact that 3 of the 4 SCT patients developed new vessels within 3 years of diagnosis of diabetes compared to 4 of 23 patients with normal hemoglobin [37] .…”

“…Proliferative sickle cell retinopathy is commonly associated with sickle cell beta thalassemia or sickle cell disease [36] , but there are reports of such in individuals with the SCT. A significant positive correlation was found between the severity of conjunctival vasculopathy and the percentage of sickle hemoglobin in 65 subjects with various sickle cell hemoglobinopathies, which included SCT [38] .…”

“…The 24-year-old male with splanchnic venous thrombosis [6] had low red cell indices with borderline HBA 2 (MCV 76.7 fl, MCH 24.7 pg, HbA 2 3.3%, HbS 35.8%, HbA 54.5%, HbF 0.8%). The patient with central retinal artery occlusion was a 14-year-old Arab Asian [32] with the following parameters (HbA 2 3.86%, HbA 58% and HbS 38.23%), while the results of the 29-year-old black woman with proliferative sickle cell retinopathy and gestational diabetes [36] showed HbA 2 5.0%, HbS 35% and HbA 53%. Though none of the cases had the required HbS concentration of >50% expected in Sβthal, it should also be noted that they also had HbA levels below 60%.…”

A retinopathy in young patient with co-inheritance of heterozygous alpha + −thalassemia and sickle trait: a case report

“…Sickle cell trait has no effect on haemoglobin concentrations [7], and heterozygous (−α/αα) α + thalassemia is associated with moderate reductions in both Mean Corpuscular Volume and haemoglobin concentration [3]. The retinopathy is an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hypertension, diabetes mellitus, syphilis, tuberculosis and sarcoidosis [8–10]. But occurring more frequently in patients with the most clinically significant haemoglobinopathies: the SC, the S-thalassemia and the SS and after 20 year-old [9, 10].…”