Hyperhaemolysis syndrome (HHS) is a rare, severe complication of transfusion characterised by a paradoxical drop in haemoglobin (Hb) levels post-transfusion, often below pre-transfusion values. Most reported cases are in patients with sickle cell disease and it has been rarely reported in other haemoglobinopathies. We report a case of HHS in a newly diagnosed delta-beta thalassaemia patient, manifesting as macrophage activation syndrome (MAS), successfully managed with intravenous immunoglobulin (IVIG), corticosteroids, rituximab, and erythropoietin. A 19-year-old female presented with fever, jaundice, and hepatosplenomegaly three weeks after a blood transfusion. Investigations revealed microcytic anaemia, elevated LDH and indirect bilirubin, and HPLC findings suggestive of delta-beta thalassaemia. She developed haemolysis and a paradoxical Hb drop after subsequent transfusions, along with reticulocytopenia. Alloimmunisation was initially absent but anti-E and anti-K antibodies were subsequently detected. Despite antigen-matched transfusions, haemolysis persisted, raising suspicion for HHS. She was managed with IVIG, methylprednisolone, erythropoietin, and rituximab, resulting in initial improvement in haemoglobin levels, which subsequently declined again. She then developed fever and leukocytosis without evidence of infection, and a high HScore raised suspicion for macrophage activation syndrome (MAS). Dexamethasone was initiated, leading to defervescence, normalisation of the reticulocyte count, and a significant rise in haemoglobin. She was discharged in stable condition and remained well at 6-month follow-up. This case highlights hyperhaemolysis syndrome (HHS) as a potential complication in patients with haemoglobinopathies. Although its exact pathogenesis remains unclear, macrophage activation appears to play an important role. Early recognition and timely initiation of appropriate immunotherapy can lead to favourable outcomes.
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