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Title: Haemophagocytic lymphohistiocytosis in a case of T cell lymphoma with gastrointestinal bleeding
e-poster Number: EPOS 31
Category: e Poster
Author Name: Dr. Pareekshith R Rai
Institute:
Co-Author Name:
Abstract :
A 13-year-old boy who was a known case of extra-nodal NK/T- cell lymphoma of the orbit on Vincristine-Asparaginase and Prednisolone chemotherapy was admitted with febrile neutropenia. He suddenly developed multiple episodes of haematochezia with compensated shock (4 g% Hb drop). He was resuscitated, transfused blood and started on inotropes. Upper GI endoscopy showed an esophageal diverticulum. Colonoscopy showed altered blood with active ooze from the IC junction. CT abdominal angiography showed active contrast extravasation in the terminal ileum. Angiography showed active extravasation from a branch of the ileocolic artery which was selectively cannulated and embolized with 10% glue. Post procedure he again developed hematochezia and also had severe thrombocytopenia with elevated Ferritin levels (2584 ng/ml). He was diagnosed to have Hemophagocytic Lymphohistiocytosis. He was taken up for surgery but during intubation he developed haematemesis and scopy showed perforation of the esophageal diverticulum for which primary repair was done. Enteroscopy did not show any ooze and the terminal ileum was empirically resected. Post-surgery he had persistent haematochezia but was clinically stable. CT angiography did not show any abnormality. He was managed conservatively but had multiple episodes of hematochezia and recalcitrant thrombocytopenia. In view of the poor prognosis, his parents decided to pursue comfort care and take him home against medical advice. Unfortunately, he passed away on his way home. Hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory syndrome characterised by pathologic activation of cytotoxic T lymphocytes and macrophages. GI bleeding can rarely occur with HLH due to transmural infiltration by lymphohistiocytes with localized ulcers and diffuse mucosal infiltration. Management of GI bleeding is challenging due to the high recurrence and mortality rate. Angioembolization and emergency surgery have been only partially successful in this condition. This case report highlights the need for us to better understand this entity and develop effective treatments for it.
