P2538 - A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in a Patient With Multiple Myeloma

Taeyang Park, MD¹, Toni-Marie Chandler, MD¹, Colton Smith, DO¹, Anish V. Patel, MD^2
1Rutgers University-Robert Wood Johnson University Hospital, New Brunswick, NJ; ²Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation which is often life-threatening. It is commonly triggered by infections like Epstein-Barr Virus (EBV). Since HLH is potentially a fatal condition, physicians must maintain a high index of suspicion in patients with nonspecific symptoms and laboratory values such as elevated liver enzymes and inflammatory markers.

Case Description/Methods: A 47-year old male with recently diagnosed multiple myeloma presented with fever and anorexia. On admission, lab tests showed cytopenia with absolute neutrophil count of 920 per µL, hemoglobin of 9.3 g/dL, and elevated liver enzymes, notably alkaline phosphatase of 1091 U/L and bilirubin of 8.2 mg/dL. He had no history of liver disease and denied alcohol use. MRI of abdomen and MRCP showed hepatosplenomegaly but normal biliary system and patent hepatic vasculature. Serologic work up for liver disease was unrevealing. As patient continued to have rising liver enzymes and persistent fevers without a clear etiology, liver biopsy was performed which revealed EBV-encoded small RNA positive (EBER) lymphohistiocytic infiltrates (Figures A, B). Further work up showed EBV DNA level of 9550 IU/mL and ferritin of 10,379 ng/mL. Bone marrow aspirate smear showed hemophagocytic histiocytes (Figure C). Diagnosis of HLH was made as patient met at least 5 of 8 criteria based on HLH-2004 protocol with fever, splenomegaly, cytopenia, hemophagocytosis, and elevated ferritin level. Treatment with rituximab and steroids was started and patient had improvement of liver enzymes and EBV viral load.

Discussion: We present a case of EBV-associated HLH in a patient with multiple myeloma. HLH is a rare disease associated with widespread immune activation leading to multiorgan failure. It can be caused by underlying genetic etiology or be associated with other disease processes such as infections (typically EBV), malignancies, or autoimmune diseases. Treatment of HLH includes a variety of potent immunosuppressive regimens including steroids and etoposides. With EBV-associated HLH, rituximab can be used. HLH can be challenging to diagnose due to its variable presentations. This case demonstrates the importance of keeping HLH as a differential for elevated liver enzymes and early consideration of liver biopsy in these clinical situations. Early identification of the disease and its etiology is essential as the condition carries a high mortality rate and therapy involves treating the underlying cause.



Disclosures:


Taeyang Park, MD¹, Toni-Marie Chandler, MD¹, Colton Smith, DO¹, Anish V. Patel, MD². P2538 - A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in a Patient With Multiple Myeloma, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.