S1724 Rare Case of Fulminant Gastrointestinal Langerhans Cell Histiocytosis Following SARS-COV-2 Infection

Abstract

Introduction: Langerhans cell histiocytosis (LCH) is a rare disease that can have either single or multi-organ system involvement. GI involvement in adults is rare, and it is typically diagnosed in asymptomatic patients during a routine screening colonoscopy. Reports of viral infections have been found to play a role in pathogenesis. In the midst of COVID-19 pandemic, there is concern that the over inflammatory response seen in response to SARS-CoV-2 infection can trigger or worsen immune mediated disorders. Case Description/Methods: Our patient is a 69-year-old male with a history of LCH who presented with copious amounts of diarrhea three weeks after a COVID infection. In terms of her LCH, she had been treated with vinblastine and prednisone and post treatment PET (Image 1A) demonstrated near complete remission. At the outside hospital, her infectious work up was nonrevealing. EGD and colonoscopy revealed multiple nonbleeding duodenal ulcers, an area of friable mucosa with shallow ulceration in terminal ileum and multiple small diverticula in sigmoid colon. Rectum, sigmoid colon, descending colon, and transverse colon were spared concerning Crohn's disease. Thus, patient was treated with intravenous steroids. However, duodenum, terminal ileum and ascending colon ultimately revealed pathology consistent with LCH. She was discharged on a steroid course. Shortly after discharge, the patient represented to following a decline in her functional status with worsening diarrhea and anasarca. A repeat PET scan demonstrated multifocal areas of disease in the liver, spleen, bone, and distal small bowel through the colon (Image 1B). She completed a 5-day course of cytarabine. She had clinical deterioration leading to hypovolemic shock and acute respiratory failure requiring pressor support and mechanical ventilation. Ultimately, the family ultimately chose compassionate extubation and the patient expired. Discussion: The purpose of this case report is to highlight that SARS-CoV-2 can potentially be a trigger for worsening of LCH. Our patient is unique in a sense that this patient’s developed fulminant GI involvement from her LCH after a recent SARS-CoV-2 infection which is known to cause an inflammatory state and gastroenteritis. This case demonstrates the need to keep rare disease such as GI involvement from LCH on the differential in patients presenting with diarrhea post SARS-CoV-19 infection.Figure 1.: Anorectal Manometry Data with CT evidence of Rectal Perforation.