HEMOPHAGOCYTOSIS - A POTENTIAL COMPLICATION IN SMALL-BOWEL TRANSPLANTATION

Background. Acquired hemophagocytosis is a histiocytic proliferation a ssociated with phagocytosis of the hemopoietic elements resulting in f ever, profound pancytopenia, hepatosplenomegaly, lymphadenopathy, and coagulopathy. Acquired hemophagocytic syndrome is a specific entity th at can be differentiated from malignant histiocytosis and familial hem ophagocytosis. It has been associated with a variety of disorders, inc luding immunodeficiency, infection, hemopathies, cancer, and autoimmun e 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 pati ent with systemic lupus erythematosus. Results. Hemophagocytosis secon dary to systemic lupus erythematosus has been reported to have a bette r response to steroid therapy than that associated with infection. In this case, the association of an autoimmune disease, high levels of im munosuppression, and posttransplant sepsis may have triggered the onse t of hemophagocytosis. Conclusion. It is suggested that patients with systemic lupus erythematosus may represent a high-risk group for devel oping hemophagocytosis after small bowel transplantation.