S2272 A Rare Presentation of GI Bleeding Associated With Hemophagocytic Lymphohistiocytosis

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease caused by impaired function of cytotoxic T cells and NK cells that leads to hemophagocytosis. It is common in children and rarely seen in adults. Gastrointestinal bleeding (GIB) associated with HLH is extremely rare. We present a patient who has acute massive gastrointestinal bleeding associated with HLH. Case Description/Methods: A 70-year-old male with history of gout, started on allopurinol recently presented with jaundice (total, conjugated bilirubin, 23.8,13.7). After negative laboratory work up, liver biopsy showed occasional hemophagocytosis within foamy macrophages, perivenular hepatocanalicular cholestasis with feathery degeneration of hepatocytes, diffuse lobular edema with fibrosis, mixed portal inflammation with lobular macrophages and increased iron deposition within hepatocytes. He was thought to have drug induced cholestatic liver injury and allopurinol was stopped. The patient continued to have jaundice with high grade fevers, anemia, neutropenia and altered mental status. Further investigations revealed ferritin of >10,000 and CMV viremia prompting initiation with Ganciclovir. Bone marrow biopsy revealed hypercellularity with profound granulocytic hypoplasia, erythroid hyperplasia, adequate megakaryocytes, abundant macrophages and occasional with erythrophagocytosis and leukophagocytosis, and negative viral immunostaining and malignancy. He was started on a dexamethasone taper. The plan was to start dose adjusted etoposide when bilirubin improved; however, the patient developed a massive GIB with hemorrhagic shock with no extravasation on CT angiogram. Endoscopy was not done due to limited benefit in HLH. Family decided to pursue comfort care due to poor prognosis. Discussion: GIB associated with HLH is due to transmural lymphohistiocytic infiltration of macrophages resulting in localized ulcers or diffuse mucosal irritation in the gastrointestinal tract. Management of GIB is challenging, owing to difficulty in localizing mucosal bleeding and increased recurrent bleeding upon intervention, and thus has extremely high mortality. Guo et al conducted a study that estimated a 12.2% rate of having a GIB as a complication of HLH, with an associated mortality of 66%. Early diagnosis of HLH and treatment is the key. Emergent surgery or angioembolization for GIB has been partially successful in a few patients. We present a patient with secondary HLH complicated with GIB and multiorgan failure, despite early treatment.