Hingorani M, Bentley C R, Jackson H, Betancourt F, Arya R, Aclimandos W A, Bird A C
Moorfields Eye Hospital, London, UK.
Eye (Lond). 1996;10 ( Pt 3):338-42. doi: 10.1038/eye.1996.70.
Retinopathy associated with sickle-C and sickle cell disease is well described. Sickle trait and haemoglobin C trait are generally considered benign conditions, with infrequent systemic manifestations. Rare cases of retinopathy in sickle trait, in the presence of contributory factors, exist and we recently reported three such patients. The occurrence of retinopathy in haemoglobin C trait is even less well documented. Haemoglobin C does not cause red blood cell sickling but is known to decrease erythrocyte plasticity and increase blood viscosity. We report three cases in which haemoglobin C trait was associated with significant peripheral vascular occlusion and seafan formation (confirmed by fluorescein angiography) similar to that seen in sickle retinopathy. Two patients had coexistent systemic disease (hypertension and diabetes mellitus). Vitreous haemorrhage was the presenting feature in two patients. It is evident that haemoglobin C trait may be associated with sight-threatening complications.
与镰状细胞C病和镰状细胞病相关的视网膜病变已有充分描述。镰状细胞性状和血红蛋白C性状通常被认为是良性病症，全身表现不常见。存在一些在有促成因素情况下镰状细胞性状发生视网膜病变的罕见病例，我们最近报告了3例这样的患者。血红蛋白C性状发生视网膜病变的情况记录更少。血红蛋白C不会导致红细胞镰变，但已知会降低红细胞可塑性并增加血液粘度。我们报告了3例血红蛋白C性状与严重外周血管闭塞和海扇状形成（经荧光素血管造影证实）相关的病例，这与镰状细胞视网膜病变所见相似。2例患者同时存在全身性疾病（高血压和糖尿病）。2例患者以玻璃体积血为首发症状。显然，血红蛋白C性状可能与威胁视力的并发症相关。
