P2536 - From Baby Joy to Liver Ploy: Ante-Partum HLH

Jonathan Selzman, DO¹, Grace Hopp, DO, MBA², Lisa Pedicone, PhD³, Carmen Landaverde, MD⁴, Eric Lawitz, MD⁴, Jan Petrasek, MD, PhD⁵, Fred Poordad, MD⁴, Fabian Rodas, MD⁴, Vipulkumar Prajapati, MD¹, Eugenia Tsai, MD^4
1University of Texas Health Science Center at San Antonio, San Antonio, TX; ²UT Health San Antonio, San Antonio, TX; ³Texas Liver Institute, San Antonio, TX; ⁴UT Health San Antonio, Texas Liver Institute, San Antonio, TX; ⁵UT Health San Antonio, Texas Liver Institute, Jackson, MS

Introduction: Hemophagocytic lymphohistiocytosis (HLH) presents with hepatitis in 30-50% of cases. We present a case of severe acute liver injury in the setting of ante-partum HLH.

Case Description/Methods: A 27-year-old female at 39 weeks gestation presented with dyspnea. Vitals and physical exam were unremarkable. Lab values were significant for AST 176 U/L, ALT 160 U/L, ALP 334 U/L, Tbili 0.6 mg/dL and INR 1.0. Urinalysis showed trace protein thus due to concerns for early preeclampsia labor was induced and patient had an uncomplicated delivery.

On day four, patient became febrile and hypotensive, requiring pressor support. Labs were notable for cytopenia (Table 1) and liver tests peaked at AST 5,234 U/L, ALT 1,578 U/L, ALP 267 U/L, Tbili 6.4 mg/dL and INR 1.6. Fibrinogen was low and ferritin was high (Table 1). Infection, viral and autoimmune causes of liver injury were ruled out. Acute fatty liver of pregnancy, intrahepatic cholestasis of pregnancy and thrombotic thrombocytopenic purpura were excluded based on lack of clinical and lab findings. Four-phase CT of the liver demonstrated hepatomegaly with increased echogenicity, patent hepatic vasculature and a normal sized spleen. Liver biopsy showed macrovesicular steatosis, lobular inflammation with mild portal inflammation (Figure 1a) without significant fibrosis. CD25, a marker for regulatory CD4+ T cells, was elevated (Table 1). Bone marrow aspirate revealed normocellular marrow but numerous histiocytes and hemophagocytosis (Figure 1 b). Patient’s clinical status and lab value improved with initiation of etoposide, dexamethasone and cyclosporine.

Discussion: HLH is a rare, life-threatening syndrome characterized by excessive inflammation and tissue destruction due to abnormal immune activation. Nearly all patients with HLH have hepatic dysfunction related to uncontrolled macrophage activity and overproduction of cytokines. Liver biopsy findings are nonspecific including sinusoidal dilatation, hepatocellular necrosis, endothelialitis, and steatosis. Diagnosis of HLH requires presence of five of nine entities (Table 1).

Ante-partum HLH is even more unusual. Pregnancy-related physiological immunomodulation and suppression of immune cells may contribute to unregulated activation and cytokine release. Due to nonspecific lab abnormalities, HLH diagnosis is often delayed. Severe acute liver injury in an ante-partum patient should prompt evaluation for HLH to ensure timely diagnosis as mortality is very high without treatment.



Disclosures:


Jonathan Selzman, DO¹, Grace Hopp, DO, MBA², Lisa Pedicone, PhD³, Carmen Landaverde, MD⁴, Eric Lawitz, MD⁴, Jan Petrasek, MD, PhD⁵, Fred Poordad, MD⁴, Fabian Rodas, MD⁴, Vipulkumar Prajapati, MD¹, Eugenia Tsai, MD⁴. P2536 - From Baby Joy to Liver Ploy: Ante-Partum HLH, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.