Elmezughi Khaled, Ekpebegh Chukwuma
Department of Internal Medicine, Walter Sisulu University, Mthatha, South Africa.
Pan Afr Med J. 2019 Aug 7;33:288. doi: 10.11604/pamj.2019.33.288.18873. eCollection 2019.
Hereditary spherocytosis is a rare cause of chronic haemolytic anaemia. It is rarer in the black population with extremely few cases reported. Initial assessment of a patient with suspected disease should include documenting clinical features of chronic haemolysis and a family history. Its management in pregnancy is challenging and needs a multidisciplinary team. We report on a black South African woman with hereditary spherocytosis and massive splenomegaly presenting with severe haemolytic anaemia at 23 week of pregnancy. She had a successful splenectomy.
遗传性球形红细胞增多症是慢性溶血性贫血的罕见病因。在黑人人群中更为罕见,报道的病例极少。对疑似该病患者的初步评估应包括记录慢性溶血的临床特征和家族史。其妊娠期间的管理具有挑战性,需要多学科团队。我们报告了一名南非黑人女性,患有遗传性球形红细胞增多症且脾肿大,在妊娠23周时出现严重溶血性贫血。她接受了成功的脾切除术。