SHM Converge Scientific Abstract ePoster Gallery-A Difficult but "Don't Miss" Diagnosis: Liver Biopsy Supported Hemophagocytic Lymphohistiocytosis Occuring After Liver Transplant

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This case describes a 53-year-old woman, three years after liver transplantation, who developed four months of worsening abdominal pain, vomiting, jaundice, and elevated liver tests. Initial liver biopsies showed inflammation and cholestasis without rejection, and ERCP removed a bile duct stone and placed a stent, but her condition continued to worsen.<br /><br />At the outside hospital, she had elevated AST/ALT, marked hyperbilirubinemia, mild cytopenias, high ferritin, elevated triglycerides, and bone marrow hemophagocytosis. She was CMV and EBV negative but positive for parvovirus B19, so the working diagnosis was drug-induced liver injury versus HLH, and she received IVIG for presumed parvovirus infection.<br /><br />At Barnes Jewish Hospital, her clinical and laboratory status deteriorated significantly: liver enzymes and bilirubin rose further, hemoglobin, white blood cell count, and platelets fell, ferritin increased dramatically, and soluble IL-2 receptor was elevated. Liver biopsy was highly informative and supported the diagnosis of hemophagocytic lymphohistiocytosis (HLH). Her H-score was 161, consistent with HLH.<br /><br />The presentation highlights the difficulty of diagnosing HLH, especially after liver transplantation, where infection, malignancy, immunosuppression, and graft-related complications can mimic it. The case emphasizes the importance of early clinical suspicion, using adult HLH tools such as the H-score, performing broad infectious testing, and considering liver biopsy as a high-yield diagnostic test.<br /><br />Ultimately, the patient was diagnosed with HLH and treated with dexamethasone and etoposide.<br /><br />Key learning points:<br />- HLH should be considered in transplant patients with progressive cytopenias, hyperferritinemia, and liver dysfunction.<br />- H-score is useful in adults; HLH-2004 criteria are pediatric-based.<br />- Liver biopsy can provide crucial diagnostic evidence.<br />- Prompt recognition and treatment are essential.

Asset Subtitle

Kamal Kolluri

Meta Tag

Author List Jason Pan, Kamal Kolluri, Xiuli Liu

Category Clinical Vignettes

Concept Secondary hemophagocytic lymphohistiocytosis

Concept H-score

Concept Post-Transplant Patient

Concept Systemic Inflammation

Concept Organ Dysfunction

Distinguished Non-Finalist

Presenter Organization Washington University School of Medicine

Presenting Author Kamal Kolluri

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

liver transplantation

hyperferritinemia

cytopenias

jaundice

liver biopsy

H-score

parvovirus B19

etoposide

Secondary hemophagocytic lymphohistiocytosis

H-score

Post-Transplant Patient

Systemic Inflammation

Organ Dysfunction