Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome. It is uncommon in the postpartum period and frequently under-recognised. Described here is a 31-year-old woman who developed HLH shortly after an uncomplicated emergency caesarean section. Initial postpartum pyrexia was attributed to Staphylococcus aureus bacteraemia and treated with intravenous antibiotics. Extensive investigations failed to identify an infectious source. An H-score of 216 indicated a high likelihood of HLH, and treatment with intravenous methylprednisolone followed by anakinra resulted in clinical stabilisation and full recovery. This case highlights HLH as an important differential diagnosis in postpartum patients presenting with unexplained fever and haematological abnormalities. Early recognition and prompt initiation of immunosuppressive therapy are critical to improving outcomes in this potentially fatal but treatable condition.
Cooke et al. (Sun,) studied this question.