Abstract
Fever with cytopenias and splenomegaly raises concern for hematologic malignancy and hyperinflammatory syndromes such as hemophagocytic lymphohistiocytosis (HLH). Distinguishing these entities is critical, as management strategies differ significantly. In this report, we describe a 19-year-old male with autoimmune hepatitis–primary sclerosing cholangitis overlap who presented with one year of recurrent fevers, cytopenias, and splenomegaly. Extensive infectious and malignant workup, including PET imaging and bone marrow biopsy, were negative. HLH was considered; however, laboratory findings were inconsistent, including ferritin of 191 ng/mL and fibrinogen of 683 mg/dL. His course remained chronic and stable. The presentation was attributed to autoimmune liver disease with systemic inflammation and hypersplenism. Distinguishing immune activation from hyperinflammation is critical to avoid unnecessary HLH-directed therapy and guide high-value hematologic care.
Recommended Citation
Firestone, MD, MS, Reed
(2026)
"Fever, Cytopenias, and Splenomegaly: A Hematologic Approach to Suspected HLH in Autoimmune Liver Disease,"
The Medicine Forum: Vol. 27, Article 28.
Available at:
https://jdc.jefferson.edu/tmf/vol27/iss1/28
