P23 Recurrent Hyperhaemolysis

Abstract

Hyperhaemolysis is a rare complication of blood transfusion in patients with SCD. An 8 year‐old boy with SCD was on a regular transfusion programme because of right frontal lobe ischaemia. He was admitted to hospital 5 days after receiving transfusion with Hb 6.5 g dL−1, reticulocyte count 330 × 109 L−1 and total bilirubin 84 μmol L−1. Haemoglobinuria was demonstrated. He was treated with oral hydration, ibuprofen and morphine for a vaso‐occlusive crisis. His Hb continued to drop to 4.1 g dL−1, reticulocyte count to 170 × 109 L−1.The DAT was negative and no red cell alloantibodies were identified. A diagnosis of hyperhaemolysis syndrome was made and he was commenced on IVIg 1 g kg−1 for 2 days and prednisolone 1 mg kg−1 for 1 week. His Hb gradually improved over a week to 6.2 g dL−1 and he did not receive transfusions. Six months later he was re‐started on a transfusion programme because of nocturnal hypoxaemia. A week after the first transfusion he was admitted with a history of lower limb and abdominal pain.Hb 7.8 g dL−1, reticulocyte count 297 × 109 L−1 and total bilirubin 106 μmol L−1 and LDH 6000 IU L−1. The Hb dropped to 3.2 g dL−1, reticulocyte count further dropped to 168 × 109 L−1 with evidence of haemoglobinuria. He developed cardiorespiratory failure requiring transfer to intensive care unit. He was given IVIg 0.5 g kg−1 for four days and three doses of methylprednisolone 4 mg kg−1 and received two RBCs units. DAT and red cell alloantibody screen were negative. He also received Erythropoietin 500 units subcutaneously three times a week. He improved over 10 days and at discharge had Hb 7.1 g dL−1, reticulocyte count 220, LDH 2033 ΙU L−1 and total bilirrubin 20 μmol L−1. He is currently on hydroxycarbamide with a stable Hb and no evidence of red cell alloantibodies. We report a case of recurrent hyperhaemolysis which is extremely rare as there were only two cases reported in the literature. The first was an adult SCD patient who had a recurrent hyperhaemolysis 27 months after the initial episode. The second was a paediatric SCD patient who had three separate episodes of hyperhaemolysis. Second and third episodes occured when additional transfusion were given 6 and 12 months after the original episode. Hyperhaemolysis may recur. It is not possible to predict which patient may recur after receiving blood. In life threatening recurrent hyperhaemolysis, use of IVIg and steroids may be life saving.