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Massive haemolysis and erythrophagocytosis in Gram‐negative sepsis

A 71-year-old woman with a history of hypertension, diabetes mellitus and chronic kidney failure was admitted to our emergency department with progressive abdominal pain for 1 d. She had no fever and was haemodynamically stable, but developed severe jaundice overnight and started to produce clear red urine. Laboratory results showed severe haemolysis with an indirect bilirubin of 214 lmol/l and a lactate dehydrogenase of 6274 iu/l. A blood film was performed, which showed massive erythrophagocytosis by neutrophils (top), abundant spherocytosis and incidental ghost cells (bottom). The neutrophils were vacuolated and showe toxic granulation with D€ ohle bodies. No fragments were visible and a direct antiglobulin test was negative using anti-IgG and anti-C3b/d antibodies, as well as anti-IgA and anti-IgM. Intravascular haemolysis due to severe sepsis was suspected, so broad-spectrum antibiotics were started. A computerized tomography scan showed no intra-abdominal pathology apart from a slight distension of the common bile duct. The patient was admitted to the intensive care unit for inotropes and respiratory support but died of overwhelming septic shock within 12 h. Post-mortem blood cultures revealed a Plesiomonas shigelloides and Escherichia coli bacteraemia. Autopsy showed haemorrhagic changes in the stomach, spleen and lungs. Several small abscesses in the liver were identified as the most likely source of the sepsis and cultures of these lesions showed the same pathogens that caused the bacteraemia. Erythrophagocytosis by circulating neutrophils is a rare phenomenon. It can be triggered by several mechanisms, including autoimmune haemolysis, spider venoms, quinine administration, certain types of leukaemia and bacteraemia. Clostridium perfringens toxins are notorious for causing massive haemolysis with very high mortality rates. Plesiomonas shigelloides and E. coli are Gram-negative bacilli that are known causes of sepsis. Both species are able to demonstrate haemolytic activity in vitro. Gram-negative sepsis can induce massive intravascular haemolysis with erythrophagocytosis. A peripheral blood film can help to identify this rare complication.