Noctural Hemoglobinuria

Introduction: Due to advances in modern medicine specialists working in obstetrics are increasingly faced with parturients with orphan diseases such as Paroxysmal nocturnal hemoglobinuria (PNH). The method of delivery in most cases is elective caesarean section (CS). However, the choice of anesthesia is still controversial.

Case report: A 25 year old primigravida presented for elective CS at 38 weeks gestation. Her history included aplastic anemia diagnosed in 1999, a recurrence in 2007, and now in remission, genetic thrombophilia; a prothrombin mutation FIIThr 165Met (het), chronic viral hepatitis B without DNA replication phase, moderate polyhydramnios, RH-negative blood (immunization at 28 weeks). Our clinical observation revealed that pregnancy together with pre-eclampsia triggered the PNH manifestation and activation of the complement system. The clinical onset of PNH portrayed itself as acute renal failure together with signs of haemolysis. These clinical signs caused significant confusion with the differential diagnoses between relapse of somatic pathology and pre-eclampsia. Platelets were transfused preoperatively. The CS was performed under general anaesthesia.

Discussion: This clinical case demonstrates the need for and the importance of early diagnosis of PNH in parturients. Parturients with PNH are one of the most complex group of pregnant women due to the potential development of obstetric and anaesthetic complications. This requires constant monitoring of clinical and laboratory parameters and a multi-disciplinary team approach involving haematologists, obstetricians and anaesthetists in the peri-operative management of the patient.