P2927 - Hemophagocytic Lymphohistiocytosis in a Liver Transplant Recipient

Sarang Thaker, MD, MS¹, Gregory Pajot, MD², Krutika Lakhoo, MD², Rohit Agrawal, MBBS², Elie Ghoulam, MD, MS², Christine Chan, MD^2
1University of Illinois at Chicago College of Medicine, Chicago, IL; ²University of Illinois at Chicago, Chicago, IL

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disorder that can be inherited or acquired, commonly from infections. Rarely, HLH can be triggered from solid organ transplantation. Patients may present with fevers, hepatosplenomegaly, rash, and neurologic changes. We present a liver transplant recipient who developed HLH secondary to transplantation.

Case Description/Methods: A 53-year-old male with Crohn’s colitis and primary sclerosing cholangitis with cirrhosis underwent orthotopic liver transplantation and was admitted six weeks later for acute kidney injury and fevers with abdominal pain. Cross-sectional imaging showed an intraabdominal hematoma for which a drain was placed, and fluid studies grew Enterococcus faecium. Despite treatment, pyrexia persisted and pancytopenia developed. Infectious studies were notable for slightly positive strongyloides antibody which was treated with ivermectin. Peripheral blood smear did not show blasts or hemolysis. Ferritin was elevated to > 15000 mcg/L. Days later, the patient seized and was intubated. Bone marrow biopsy showed numerous histiocytes and hemophagocytosis without fungal or viral infections. Serum interleukin-2 (IL-2) and triglyceride levels were elevated; and a diagnosis of HLH was confirmed. In evaluation of triggers, a comprehensive workup as shown in Table 1 was negative. The etiology of HLH was ultimately deemed secondary to organ transplantation.



The patient was initially treated with dexamethasone followed by several cycles of etoposide, and finally emapalumab due to refractory disease. The patient also developed gastrointestinal bleeding refractory to transfusions. Upper endoscopy revealed diffuse duodenal mucosal denudation and bleeding (Figure 1) with biopsies demonstrating shortened villi without features of graft-versus-host disease or strongyloides hyperinfection. He subsequently developed shock secondary to multi-drug resistant bacterial and fungal infections. An evaluation for allogenic stem cell transplant was conducted, but ultimately the patient succumbed to overwhelming infections. Liver graft function remained intact until the development of septic shock.

Discussion: HLH is characterized by high fevers, hyperferritinemia, hypertriglyceridemia, and elevated IL-2 levels. Bone marrow biopsy is necessary for diagnosis and will show hemophagocytosis. Treatment is with immunosuppression or chemotherapy. HLH is often fatal and prompt recognition and consultation with Hematology is necessary.




Disclosures:


Sarang Thaker, MD, MS¹, Gregory Pajot, MD², Krutika Lakhoo, MD², Rohit Agrawal, MBBS², Elie Ghoulam, MD, MS², Christine Chan, MD². P2927 - Hemophagocytic Lymphohistiocytosis in a Liver Transplant Recipient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.