Hemophagocytosis: A potential complication in small bowel transplantation

Background. Acquired hemophagocytosis is a histiocytic proliferation associated with phagocytosis of the hemopoietic elements resulting in fever, profound pancytopenia, hepatosplenomegaly, lymphadenopathy, and coagulopathy. Acquired hemophagocytic syndrome is a specific entity that can be differentiated from malignant histiocytosis and familial hemophagocytosis. It has been associated with a variety of disorders, including immunodeficiency, infection, hemopathies, cancer, and autoimmune diseases, as well as with the period following solid organ and bone marrow transplantation. Methods. We describe a case of a fatal outcome, due to hemophagocytosis, after small bowel transplantation in a patient with systemic lupus erythematosus. Results. Hemophagocytosis secondary to systemic lupus erythematosus has been reported to have a better response to steroid therapy than that associated with infection. In this case, the association of an autoimmune disease, high levels of immunosuppression, and posttransplant sepsis may have triggered the onset of hemophagocytosis. Conclusion. It is suggested that patients with systemic lupus erythematosus may represent a high-risk group for developing hemophagocytosis after small bowel transplantation.