Immune-mediated Coombs negative intravascular haemolysis in systemic lupus erythematosus (SLE)

A 27-year-old woman presented with a history of excessive hair loss, loss of appetite, loss of weight, amenorrhoea and loss of axillary and pubic hair for 6 months followed by fever and vomiting for 5 months and abdominal pain for 1 month. During the course of her illness, the patient developed intravascular haemolysis as evidenced by a drop in haemoglobin, indirect hyperbilirubinaemia, raised lactate dehydrogenase (LDH) and haemoglobinuria. Examination revealed severe pallor, mild icterus, elevated jugular venous pressure, generalised lymphadenopathy and hyperpigmentation. Investigations revealed severe anaemia, indirect hyperbilirubinaemia, raised LDH and negative Coombs test. Antinuclear antibody and anti-dsDNA, anti-Sm and anti-SS-A/Ro antibodies were positive and complement C3 was low. The patient was diagnosed to have systemic lupus erythematosus and immune-mediated intravascular haemolysis and was treated with prednisolone and hydroxychloroquine. Haemolysis resolved following steroid therapy, and during follow-up, there were no further episodes of haemolysis.