Severe hereditary spherocytosis in pregnancy: A case report and multidisciplinary management strategy

Hereditary spherocytosis is a congenital haemolytic anaemia caused by defects in red blood cell membrane proteins, resulting in a spherical shape and increased splenic destruction. It is the most common hereditary haemolytic anaemia worldwide. Management becomes more complex during pregnancy due to increased physiological stress and haematological demands. We present the case of a 22-year-old woman diagnosed with HS since childhood, with a history of cholecystectomy and blood transfusions. In her second trimester, she presented with left upper quadrant pain, severe anaemia, and splenomegaly. She was admitted with tachycardia and chest pain, and active haemolysis was detected. Therapeutic red blood cell exchange was performed via apheresis, leading to clinical stabilisation and improved haematological parameters. A splenectomy was subsequently carried out. At term, the patient entered active labour and delivered vaginally without immediate neonatal complications. Postpartum, she experienced uterine atony unresponsive to medical management, which was successfully treated with a Bakri balloon. A urinary tract infection was also diagnosed and treated with antibiotics. The newborn developed jaundice and clinical signs suggestive of hereditary spherocytosis. This case highlights the importance of multidisciplinary management in pregnant patients with hereditary spherocytosis, where timely interventions such as therapeutic apheresis and splenectomy can significantly improve maternal and foetal outcomes. Close monitoring and comprehensive care are essential to prevent complications and achieve favourable results in pregnancies complicated by rare haematological conditions like hereditary spherocytosis.