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Poster Session E - Tuesday Afternoon

Category: Liver

E0554 - Acute Liver Injury as Presenting Feature of Hemophagocytic Lymphohistiocytosis in a Patient With Post-COVID 19 and EBV Infection

Tuesday, October 25, 2022

3:00 PM – 5:00 PM ET

Location: Crown Ballroom

Has Audio

Presenting Author(s)

Afshin A. Khan, MD, MPH

Cleveland Clinic Foundation
CLEVELAND, OH

Afshin A. Khan, MD, MPH, Kaitlin Payne, MD, Talal Adhami, MD
Cleveland Clinic Foundation, Cleveland, OH

Introduction: Viral infections such as Epstein Barr Virus (EBV) have been associated with secondary hemophagocytic lymphohistiocytosis (HLH) with some recent studies alluding to an association between severe COVID-19 and HLH. Herein, we report a case of a patient with an earlier mild case of COVID, followed by EBV infection, that precipitated HLH.

Case Description/Methods: A 36-year-old man with a history of mild COVID-19 infection a month prior presented to an outside hospital with nausea, fatigue, and jaundice. Patient had total bilirubin (tibili) 7.1, ALT 2714, AST 1273 and an elevated EBV viral capsid antigen. Liver US was negative. CT abdomen showed significant portal hepatitis and peri-pancreatic head lymphadenopathy. Lymph node biopsy was unrevealing. Patient was diagnosed with mononucleosis and discharged. Patient presented to multiple institutions and discharged with mononucleosis associated liver injury. With worsening jaundice, patient ultimately presented to Cleveland Clinic with tbili 23.8, conjugated bilirubin 19.8, ALT 1400, AST 1145, Alkaline phosphatase 461, with repeat EBV titers negative. CT revealed upper abdominal lymphadenopathy in peri-portal and periceliac regions. MRCP confirmed lymphadenopathy, borderline splenomegaly with no biliary dilation. Acute liver injury workup was negative. Liver biopsy showed marked lobular hepatitis, areas of dropout with hemorrhage and increased cellularity within the sinusoids and evidence of hemophagocytosis. The portal areas were expanded by inflammatory cell infiltrates including plasma cells, lymphocytes, histiocytes, and granulocytes. Patient was diagnosed with HLH, using the criteria in Table 1. H-score was 184 points with 70-80% probability of HLH. Patient was discharged with close follow up with hepatology and hematology for treatment initiation.

Discussion: HLH carries a grim prognosis and requires earlier detection and treatment. Acute phase COVID-19 has been associated with HLH, though post-COVID 19 cases associated with HLH are rare. It is thought that post-COVID 19 causes immune dysregulation and activation of macrophages which can cause HLH. Though asymptomatic, our patient subsequently tested positive for EBV, which was cleared by the time repeat testing was done. This raises the question for concurrent or subsequent infections as precipitating factors for HLH. Our case report highlights the importance of exploring HLH in patient where no obvious signs of acute liver injury are present, particularly in patients with COVID-19 infection.

Figure: Table 1. Comparison of HLH- 2004 diagnostic guidelines an H-score in our patient

HLH-2004 Criteria^*

H-Score Criteria

Fever

Present

Temperature, °F (°C)

< 101.1 (< 38.4)

101.1–102.9 (38.4-39.4) (+33)

>102.9 ( >39.4) (+49)

Splenomegaly

Present

Organomegaly

Hepatomegaly or splenomegaly (+33)

Hepatomegaly and splenomegaly (+38)

Cytopenias (affecting ³2 of 3 lineages in the peripheral blood)

< ![if !supportLists] >· < ![endif] >Hemoglobin < 90 g/L

< ![if !supportLists] >· < ![endif] >Platelets < 100 x 10⁹/L

< ![if !supportLists] >· < ![endif] >Neutrophils < 1.0 x 10⁹/L

Absent

Number of cytopenias

Defined as hemoglobin ≤9.2 g/dL (≤5.71 mmol/L) and/or WBC ≤5,000/mm³ and/or platelets ≤110,000/mm³

1 lineage

2 lineages (+24)

3 lineages (+34)

Hypertriglyceridemia and/or hypofibrinogenemia:

< ![if !supportLists] >· < ![endif] >Fasting triglycerides ³ 3.0 mmol/L (i.e. ³265 mg/dl)

< ![if !supportLists] >· < ![endif] >Fibrinogen £1.5 g/L

Absent (triglycerides 217 mg/dl; fibrinogen 2.27 g/L)

Triglyceride, mg/dL (mmol/L)

< 132.7 (< 1.5)

132.7-354 (1.5–4) (+44)

>354 ( >4) (+64)

Fibrinogen, mg/dL (g/L)

>250 ( >2.5)

≤250 (≤2.5) (+30)

Hemophagocytosis in bone marrow/spleen/lymph nodes

Present in liver biopsy

Hemophagocytosis features on bone marrow aspirate

Yes (+35)

Ferritin ³ 500 mg/L

Present (5,511.0)

Ferritin, ng/mL (or μg/L)

< 2,000

2,000–6,000 (+35)

>6,000 (+50)

Low or absent NK-cell activity

Absent (low normal)

AST, U/L

< 30

≥30 (+19)

Soluble CD25 (i.e., soluble IL-2 receptor) ³ 2,400 U/ml

Present (5803.6)

Known underlying immunosuppression

Yes (+18)

^* The diagnosis of HLH can be established if one of either 1 or 2 below is fulfilled 1) A molecular diagnosis consistent with HLH, 2) Diagnostic criteria for HLH fulfilled (5 out of 8 criteria below)

Table: Table 1. Comparison of HLH- 2004 diagnostic guidelines an H-score in our patient

Disclosures:

Afshin Khan indicated no relevant financial relationships.

Kaitlin Payne indicated no relevant financial relationships.

Talal Adhami indicated no relevant financial relationships.

Afshin A. Khan, MD, MPH, Kaitlin Payne, MD, Talal Adhami, MD. E0554 - Acute Liver Injury as Presenting Feature of Hemophagocytic Lymphohistiocytosis in a Patient With Post-COVID 19 and EBV Infection, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.