SHM Converge Scientific Abstract ePoster Gallery-Uncovering Hemophagocytic Lymphohistiocytosis in the Inpatient Setting

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The patient initially presented with chest pain, shortness of breath, fever, chills, and night sweats after recent cruise-ship travel to several Caribbean and Gulf Coast locations, including Belize and Honduras. Initial exam and routine labs were largely unremarkable, but a markedly elevated D-dimer led to CT pulmonary angiography, which showed mild mediastinitis with small mediastinal lymph nodes. Infectious testing was negative for HIV, legionella, hepatitis B, and malaria, though EBV antibodies were detected.<br /><br />Because of concern for lymphoma or tuberculosis, the patient underwent mediastinal and lymph node excision, but results were inconclusive. He then developed worsening anemia, hyperferritinemia (ferritin 10,000), and later severe transaminitis with concern for acute liver failure, prompting high-dose steroids. Bone marrow biopsy was also inconclusive and did not show lymphoma or a myeloid neoplasm. Since steroids may have affected the marrow findings, a liver biopsy was performed and showed abundant CD163/CD68-positive hyperplastic macrophages with hemophagocytosis and hepatocyte apoptosis.<br /><br />A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made based on five criteria: elevated IL-2 (10,521), ferritin 10,000, hemophagocytosis in liver tissue, fever, and splenomegaly. The diagnosis was further supported by cytopenias, including anemia, thrombocytopenia, and lymphopenia.<br /><br />The patient was treated with dexamethasone and rituximab; etoposide was withheld because of acute hepatic failure. Treatment lasted eight weeks, with weekly rituximab and dexamethasone tapered over time. He improved clinically and had resolution of axillary and mediastinal lymphadenopathy before discharge.

Asset Subtitle

Charles Wang

Meta Tag

Author List Anupsinh Uttam, Charles Wang, Quratulanne Jan, Sarika Chauhan

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Fever

Concept Cytopenia

Concept Ferritin

Concept Soluble interleukin-2 receptor

Distinguished Non-Finalist

Presenter Organization University of Texas Medical Branch

Presenting Author Charles Wang

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

ferritin 10000

hemophagocytosis

mediastinal lymphadenopathy

acute liver failure

dexamethasone

rituximab

cytopenias

splenomegaly

Hemophagocytic Lymphohistiocytosis

Fever

Cytopenia

Ferritin

Soluble interleukin-2 receptor