NewYork-Presbyterian Brooklyn Methodist Hospital Brooklyn, NY
Neil Khoury, MD1, Pratima Dibba, MD, MBA1, Irwin Grosman, MD2, Megha Kothari, MD1; 1NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY; 2New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY
Introduction: Neutropenic enterocolitis is a life-threatening diagnosis of exclusion commonly presenting with fever, abdominal pain, and radiographic evidence a thickened bowel wall (1). Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune-mediated disorder characterized by systemic inflammation, severe cytokine storms, and organ damage (2). HLH is commonly triggered by viral infection or malignancy and is associated with significant in-hospital mortality (3). It is not commonly triggered by bacterial infection and does not typically present as neutropenic enterocolitis. We present a case of a patient who presented with neutropenic enterocolitis, which may have been a cause or consequence of HLH.
Methods: A 56-year old African American male with hypertension, type 2 diabetes mellitus, end-stage renal disease on hemodialysis, cardiomyopathy, and recurrent left-sided pleural effusions, presented with sudden-onset diffuse abdominal pain associated with nausea, vomiting, and non-bloody diarrhea. On admission, he was febrile and tachypneic. On exam, he appeared to be in pain. His abdomen was nondistended with hypoactive bowel sounds. He exhibited guarding, and there was tenderness to palpation, however there was no rebound tenderness. His labs were significant for bicytopenia (leukopenia and anemia), and a lactate of 9.42 mmol/L. CT scan of the abdomen and pelvis was consistent with enterocolitis (Figure 1). Despite supportive measures with broad spectrum antibiotics and fluids, his mental status and respiratory status declined, eventually requiring an ICU level of care. Although no specific pathogen was identified, workup revealed a likely diagnosis of HLH. Patient was treated with intravenous steroids, followed by a steroid taper. Treatment with etoposide was postponed due to the patient’s renal dysfunction, rapid deterioration and death. Discussion: Neutropenic enterocolitis has yet to be documented as the initial presentation of HLH. We present a case of a patient who presented with severe neutropenic enterocolitis, who met diagnostic criteria for HLH. The overlap between diagnostic features of severe sepsis and HLH made the diagnosis of HLH challenging. Continued rapid decline and persistent leukocyte dysfunction prompted further investigation, which led to his diagnosis. Early identification of HLH in the setting of neutropenic enterocolitis may expand therapeutic possibilities and help improve outcomes for patients with this life-threatening condition.
Radiographic image revealing diffuse large bowel wall thickening
Disclosures: Neil Khoury indicated no relevant financial relationships. Pratima Dibba indicated no relevant financial relationships. Irwin Grosman indicated no relevant financial relationships. Megha Kothari indicated no relevant financial relationships.