Haemophagocytic lymphohistiocytosis as an initial presentation of undiagnosed systemic lupus erythematosus

Clinicians should suspect haemophagocytic lymphohistiocytosis in patients with persistent fever and cytopaenias, especially when laboratory findings (e.g. hyperferritinaemia, hypertriglyceridaemia) do not improve with standard treatments.Systemic lupus erythematosus may initially present as secondary haemophagocytic lymphohistiocytosis. Integration of clinical, radiological and immunological data can aid in early differentiation.