Hackensack Meridian Jersey Shore University Medical Center Neptune Township, NJ
Christopher Schreiber, MD1, Harshavardhan Sanekommu, MD2, Sobaan Taj, MD2, Jayasree Ravilla, MD3, Aidan Farrell, BS1, Pranav Shah, MD1 1Hackensack Meridian Jersey Shore University Medical Center, Neptune Township, NJ; 2Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ; 3Monmouth Medical Center/RWJBH, Long Branch, NJ
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a condition with dysregulated immune cells and low interferon-gamma, causing excessive cytokine release, organ damage. Secondary HLH can result from infections, neoplasms, or autoimmunity. Our case study describes a patient with SARS-CoV-2 and HIV infections developing secondary HLH, revealing viral-immune interplay in HLH pathogenesis.
Case Description/Methods: 24 year old male without past medical history presents with 2-month history of progressive fatigue and lethargy. He also has blood-tinged sputum, unintentional weight loss, and shortness of breath. On arrival, vitals were: 36.8 C, blood pressure of 105/67, heart rate of 104, respiratory rate of 18 with saturating 98% on 2L nasal cannula. Physical exam was pertinent for bitemporal wasting and scleral icterus. Significant labs: International normalized ratio 1.8, fibrinogen 375 mg/dL, hemoglobin 12.2 g/dL, platelets 146 x10^3/uL, white blood cell count 0.6 x 10^3/uL, creatinine 7.79 umol/L, blood urea nitrogen 98, total bilirubin 4.8 umol/L, aminotransferase 2,522 IU/L, alanine aminotransferase 787 IU/L, lactate dehydrogenase 2,314 U/L, D-dimer 6,402 mg/L, procalcitonin 153.5 ng/mL, ferritin >16,500 ng/mL, and lactic acid 1.44 mmol/L. He tested positive for COVID-19 with imaging showing bilateral lung infiltrates and antibiotics were initiated. Patient was admitted to the intensive care unit for multiorgan failure, requiring emergent hemodialysis. Pancytopenia workup revealed CD4+ count 9 and HIV viral load of 762,000. Antiretrovirals were not initiated as in multiorgan failure. He finished 3 days of remdesivir before it was stopped due to worsening liver function and continued dexamethasone for 10 days. Acute liver failure worsened with AST >6,000, ALT 1,829, and total bilirubin 7.2. He received N-acetyl cysteine with no improvement. All workup for infectious, metabolic, and genetic causes of acute liver failure was unremarkable. Subsequently, he developed severe hepatic encephalopathy, acute respiratory distress syndrome requiring intubation and pronation, and pressor requirement. With the newly diagnosed HIV and COVID19, and worsening multiorgan failure, HLH was strongly considered, and confirmed using the HLH-2004 protocol. Bone marrow biopsy showed increased histiocytes with hemophagocytosis (Image 1). Unfortunately, he ultimately succumbed to his condition.
Discussion: Our case highlights the need to consider HLH in acute liver failure/multiorgan failure of indeterminate cause.
Figure: Bone marrow specimen. A. Bone marrow biopsy showing increased number of macrophages admixed with normal bone marrow elements. Some of the macrophages have engulfed red cell (arrowhead) in their cytoplasm (A; H&E stain, 1000x). B-D. Bone marrow aspirate smears reveal increased macrophages with increased phagocytic activity - phagocytosed red cells and platelets (arrowhead) and neutrophils (arrow) (B-D; Wright-Giemsa stain, 1000x). The insets show iron stained smears highlighting iron-laden macrophages with ingested hematopoietic cells (Iron stain, 1000x).
Disclosures:
Christopher Schreiber indicated no relevant financial relationships.
Harshavardhan Sanekommu indicated no relevant financial relationships.
Sobaan Taj indicated no relevant financial relationships.
Jayasree Ravilla indicated no relevant financial relationships.
Aidan Farrell indicated no relevant financial relationships.
Pranav Shah indicated no relevant financial relationships.
Christopher Schreiber, MD1, Harshavardhan Sanekommu, MD2, Sobaan Taj, MD2, Jayasree Ravilla, MD3, Aidan Farrell, BS1, Pranav Shah, MD1. P3917 - Multiorgan Failure Including Acute Liver Failure from Hemophagocytic Lymphocytosis Secondary to HIV and COVID-19, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.