Variant Neutropenic Enterocolitis Presenting as Acute Appendicitis

Abstract

Neutropenic enterocolitis (NE) is characterized by fever, abdominal pain, and neutropenia. Observed most commonly in relation to solid, hematologic, and lymphoproliferative malignant tumors and their treatment, this disease entity may involve a wide area of the gastrointestinal tract. We report the first case of NE with isolated appendiceal involvement in a non-oncologic neutropenic patient who was managed successfully medically. In addition, we formally recognize neutropenic appendicitis as a variant of NE and differentiate it from acute non-neutropenic appendicitis. Case report and review of the pertinent English language literature. A 50-year-old man with seronegative large-joint arthritis presented emergently with a one-day history of subjective fever and acute-onset abdominal pain in the setting of recently developed sulfasalazine-induced agranulocytosis. Abdominal examination revealed mild-to-moderate tenderness and rebound tenderness in the right lower quadrant. Computed tomography (CT) of the abdomen demonstrated findings consistent with acute appendicitis. The patient improved clinically with non-operative management including a broad-spectrum antibiotic, with normalization of the white blood cell count four days after initiation of granulocyte-colony stimulating factor therapy. Given the similarity in clinical presentation and CT imaging in patients with neutropenic and non-neutropenic appendicitis, accurate recognition of neutropenic appendicitis rests on a thorough patient history and a high index of suspicion in febrile neutropenic patients.