P4719 - Secondary Hemophagocytic Lymphohistiocytosis in a Young Patient With Human Immunodeficiency Virus and Epstein-Barr Virus-Associated Classic Hodgkin Lymphoma

Chloe Hundman, MD, Christopher D.. Jackson, MD
University of Tennessee Health Science Center, Memphis, TN

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of excessive immune activation. Secondary HLH can result from malignancy, infection, or immune deficiency/autoimmune disease. Our case describes a young patient with previously undiagnosed human immunodeficiency virus (HIV) and Epstein-Barr Virus (EBV) associated Classic Hodgkin Lymphoma (CHL) developing secondary HLH.

Case Description/Methods: A 27-year-old female presented with a 2-month history of generalized weakness, shortness of breath, nausea, vomiting, anorexia, abdominal swelling, and jaundice. She also reported a 25-pound weight loss and night sweats. She left against medical advice (AMA) from another hospital two days prior after presenting with the same symptoms. At the outside hospital, her vitals on arrival were notable fever, tachycardia, hypotension, and tachypnea. Her physical exam was notable for scleral icterus and non-tender abdominal swelling. Labs revealed a positive HIV Ab/Ag, CD4 7 cells/mcL, viral load 700,000 copies/ml, ferritin 14,876 ng/mL, triglycerides 426 mg/dL, AST 159 unit/L, ALT 86 unit/L, total bilirubin 24 mg/dL, haptoglobin 473 mg/dL, CRP 269 mg/L, PT 29 seconds, INR 2.6, platelets 17 thou/mcL, and hemoglobin 5.9 g/dL. Hepatitis panel, histoplasmosis, cryptococcus, acid-fast bacillus blood cultures were all negative. Computerized tomography (CT) abdomen and pelvis revealed subcutaneous abdominal tumors, extensive lymphadenopathy in the retroperitoneum and right inguinal region, and splenomegaly. She was diagnosed with HIV, pancytopenia (requiring multiple transfusions), acute liver failure, acalculous cholecystitis, and HLH, as she met the 5/9 clinical criteria (fever, splenomegaly, peripheral blood cytopenias, hypertriglyceridemia, hyperferritinemia). Intravenous immune globulin and steroids were started, and a bone marrow biopsy was performed. She was responding well to treatment prior to AMA discharge. When she arrived at our hospital, her presentation (vitals, exam, labs) was similar to that at outside hospital. Preliminary bone marrow results showed EBV-positive CHL with histologic evidence of hemophagocytosis (Image 1). Our malignant hematology service was consulted, and she began chemotherapy along with antiretroviral therapy.

Discussion: HLH is a rare, life-threatening syndrome of excessive immune activation. HIV can be associated with HLH. However, malignancies and other infectious etiologies should be ruled out prior to attributing HLH to their immunodeficiency status.



Disclosures:


Chloe Hundman, MD, Christopher D.. Jackson, MD. P4719 - Secondary Hemophagocytic Lymphohistiocytosis in a Young Patient With Human Immunodeficiency Virus and Epstein-Barr Virus-Associated Classic Hodgkin Lymphoma, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.