A Case Report on Hyperhaemolysis Syndrome in a Patient with Sickle cell Anaemia

Aims: Increase awareness to the diagnosis of Hyperhaemolysis syndrome and prevent critical anaemia.

Presentation of Case: A 40 year old woman with sickle cell anaemia (SCA) was readmitted with symptoms suggestive of sepsis. In the previous admissions she was received 5 units of cross-matched compatible red blood cells (RBCs), and was discharged on last admission with a PCV of 24%. On readmission, she had a PCV of 13%, and was transfused 4 units of cross-matched compatible RBCs. Her PCV marginally improved to 16% but dropped to 12% the next day, with deteriorating clinical condition. 2 units of cross-matched compatible RBCs transfusions was requested with a haematologist review. A diagnosis of hyperhaemolytic syndrome was made, supported by laboratory findings of; haemoglobinuria and hyperbilirubinemia on urine analysis: negative direct antiglobulin test (DAT); negative Indirect antiglobulin test (IDAT); poor reticulocyte response with reticulocyte count of 8.3% (0.5%–2.5%), and reticulocyte production index at 1.66; Peripheral blood smear showed nucleated RBCs and spherocytes, Albumin- 32g/dl (35- 50g/dl), increased total bilirubin- 55.4mmol/L (17- 22.3 mmol/L). Further blood transfusions was suspended, and steroids commenced for 5 days. Patient’s clinical condition subsequently improved. She was discharged on the 5th day of steroid therapy with a PCV of 20%, followed by tapering doses of prednisolone for 4 weeks. One year of follow-up showed no new red blood cell antibody in her serum, no need further transfusions, and a steady state PCV of 25%.

Conclusion: Hyperhaemolysis syndrome is a potentially life threatening complication of blood transfusion. High index of suspicion and early recognition is important especially when managing patients with SCA who present with worsening anaemia after RBC transfusions.