BACKGROUND: Neutropenic enterocolitis, sometimes called typhilitis, is the most common gastrointestinal infection related to neutropenia, but its rarity, confusing terminology, and protean, non-specific manifestations result in variable approaches to diagnosis and management. METHODS: Review of pertinent English-language literature. RESULTS: The true incidence of neutropenic enterocolitis is unknown, but may be 5% or more among adult patients receiving chemotherapy for solid malignant tumors. The incidence is reported to be slightly lower in children. Estimates are made complex by recent recognition that neutropenia of any cause may be associated with enterocolitis; reports of non-chemotherapy drug-associated cases are increasing. Mortality rates are reported currently to be between 30% to 50%. The exact pathogenesis is also unknown, and may contribute to the varied nomenclature in use. Gut mucosal ulcerations may result from direct drug-related cytotoxicity, or from neutropenia itself. Microbial invasion of the bowel wall proceeds unimpeded. Pathological changes include inflammation and edema, presumably followed by ulceration, transmural necrosis, and perforation. The classic clinical presentation consists of fever, abdominal pain, and neutropenia, but diagnosis is often hindered by subtle or non-specific clinical findings, making computed tomography the linchpin of diagnosis. The wide spectrum of clinical presentation requires an individualized approach to therapy. Medical management, including administration of granulocyte colony-stimulating factor, may be appropriate for patients who do not have gastrointestinal bleeding, peritonitis, or intestinal perforation. Surgical management is generally reserved for patients who fall into any of the exceptional categories, and consists usually of bowel resection and stoma creation. CONCLUSIONS: Neutropenic enterocolitis is a heterogeneous diseazse state with the capacity to affect many areas of the gastrointestinal tract, and disease severity that ranges from mild to fatal. A high index of suspicion is needed for all patients who present with fever and abdominal pain in the setting of neutropenia. Early detection allows a majority of cases to resolve with nonoperative management and supportive care, but surgical intervention is mandatory for peritonitis, bowel perforation, or gastyrointestinal hemorrhage that persists despite correction of coagulopathy.
