Background and aims: Reactive hemophagocytic syndrome (RHS) is a rare disease in which inappropriately activated macrophages consume bone marrow-derived cells. Most cases are associated with infection in the setting of immunodeficiency. The widespread use of immunosuppressive therapy in the treatment of inflammatory bowel disease (IBD) places patients with Crohn's disease and ulcerative colitis at risk of this complication. No concerted effort has been made to alert gastroenterologists of this condition, and treatment recommendations are lacking. The aims of this study were to describe the clinical and laboratory features of RHS associated with IBD and to review diagnostic criteria, treatment options, and pathogenesis.
Materials and methods: Clinical and laboratory data were pooled from the clinical practice of the investigators and from published cases. Descriptive statistics were performed.
Results and conclusions: Seven cases of RHS complicating the treatment of IBD were identified. All patients were on immunosuppressive therapy, with nearly half taking >1 agent. All patients presented with fever, leukopenia, anemia, and hyperferritinemia. Infection by a member of the herpesvirus family or an intracellular pathogen precipitated RHS in 6 of 7 patients. The mortality rate was 29%. The diagnosis of RHS should be considered in patients with IBD taking immunosuppressive therapy who present with fever and cytopenia. Evaluation should begin with a serum ferritin. In patients with a serum ferritin > or =10,000 ng/mL, a bone marrow biopsy should be performed to confirm hemophagocytosis. If the initial evaluation is negative, then clinical suspicion should be maintained until the episode resolves.