The Not so Innocent Kissing Disease: A Case of HLH in a Crohnʼs Patient on a Thiopurine: 2016 ACG Presidential Poster Award

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal disease of immune activation. It is often triggered by a viral infection, typically Epstein-Barr Virus (EBV). We present a case of a young woman with Crohn's disease on 6-mercaptopurine (6-MP), referred from an outside hospital, with multi-organ failure that was recognized as HLH upon arrival. This case highlights the importance of early recognition and the need for a low index of suspicion for HLH in young adults on immunomodulators. A 19-year-old woman with Crohn's disease, treated with 6-MP, presented to an outside hospital with complaints of weakness, fever, and sore throat for 1 week. Her exam included orophyrangeal inflammation with tonsilar exudate and cervical lymphadenopathy. Labs revealed pancytopenia, transaminitis, and hypertriglyceridemia. She was diagnosed with EBV, which was confirmed by monospot. The patient was treated symptomatically, given antibiotics for neutropenia, and a course of steroids. However, her clinical status deteriorated and after 15 days, she was transferred to our hospital with multi-organ failure. Upon arrival, she met 5/8 diagnostic criteria for HLH (Table 1) and was started on dexamethasone, cyclosporine, and etoposide prior to send out labs that confirmed elevated CD25. EBV RNA on admission was 2 million. Despite a decrease in EBV viral load, her condition worsened and she was placed on a course of intravenous immunoglobulin. A CT scan revealed ileocolonic inflammation, believed to be neutropenic enterocolitis. Subsequently, the patient developed a colonic perforation. Despite intensive supportive measures, the patient expired on hospital day 51.Table 1: Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis (HLH)Inflammatory bowel disease (IBD) patients, especially young adults, have an increased risk of complications from viral infections while on immunomodulators such as 6-MP. Our case highlights how a simple case of infectious mononucleosis can lead to tragic results in this at risk population. The current literature consists mainly of case reports with one series documenting 7 IBD patients on a thiopurine who developed HLH. Any delay in diagnosis of HLH increases the mortality risk. As such, IBD patients on immune suppression who present with symptoms of infectious mononucleosis should be watched for signs of HLH. As an increasing number of young IBD patients transition to adult gastroenterologists, thorough investigation and a low index of suspicion can prevent this devastating sequela of EBV.