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Peer-reviewedCC BY-NC-NDJPGN reports

Disseminated histoplasmosis mimicking autoimmune hepatitis as a trigger of secondary hemophagocytic lymphohistiocytosis in a pediatric patient

  • Thomas Ratchford1
  • Talal Al Hendawi2
  • Eumenia Castro3
  • Pelin Cengiz2
  • James Conway4
  • Shelly Cook3
  • Leanne Mansfield5
  • Nicholas Pytel6
  • Christine M. Seroogy5
  • Katelyn Watkin7
  • Jo Wilson5
  • Jonathan Wong1
2 more authors
  • Therese Woodring6
  • Katryn N. Furuya1
7 affiliations
  1. 1Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition University of Wisconsin‐Madison Madison Wisconsin USA
  2. 2Department of Pediatrics, Division of Critical Care University of Wisconsin‐Madison Madison Wisconsin USA
  3. 3Department of Pathology and Laboratory Medicine University of Wisconsin‐Madison Madison Wisconsin USA
  4. 4Department of Pediatrics, Division of Infectious Diseases University of Wisconsin‐Madison Madison Wisconsin USA
  5. 5Department of Pediatrics, Division of Allergy, Immunology, and Rheumatology University of Wisconsin‐Madison Madison Wisconsin USA
  6. 6Department of Pediatrics, Division of Hematology, Oncology, Transplant, and Cellular Therapy University of Wisconsin‐Madison Madison Wisconsin USA
  7. 7Department of Pediatrics, Division of Genetics and Metabolism University of Wisconsin‐Madison Madison Wisconsin USA
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Abstract

Patients presenting with hemophagocytic lymphohistiocytosis (HLH) are often critically ill, and rapid identification of both HLH and underlying triggers in secondary HLH is essential for successful treatment. We present a case of secondary HLH activated by disseminated histoplasmosis (DH) that mimicked autoimmune hepatitis (AIH) on liver histology and briefly led to an incorrect liver diagnosis. Once DH was identified, he was treated with antifungals in addition to HLH therapies and stabilized. He was subsequently found to have a heterozygous variant of uncertain significance in STAT1. Our case is the first in the literature to describe liver biopsies in a pediatric patient who has DH that had triggered HLH, and due to striking histology that mimicked AIH, important to report.

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Cite

BibTeXRIS
BibTeX
@article{ratchford2026disseminated,
  title = {{Disseminated histoplasmosis mimicking autoimmune hepatitis as a trigger of secondary hemophagocytic lymphohistiocytosis in a pediatric patient}},
  author = {Thomas Ratchford and Talal Al Hendawi and Eumenia Castro and Pelin Cengiz and James Conway and Shelly Cook and Leanne Mansfield and Nicholas Pytel and Christine M. Seroogy and Katelyn Watkin and Jo Wilson and Jonathan Wong and Therese Woodring and Katryn N. Furuya},
  journal = {JPGN reports},
  year = {2026},
  month = nov,
  doi = {10.1002/jpr3.70296},
  url = {https://europepmc.org/article/PMC/PMC13729236},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - Disseminated histoplasmosis mimicking autoimmune hepatitis as a trigger of secondary hemophagocytic lymphohistiocytosis in a pediatric patient
AU  - Thomas Ratchford
AU  - Talal Al Hendawi
AU  - Eumenia Castro
AU  - Pelin Cengiz
AU  - James Conway
AU  - Shelly Cook
AU  - Leanne Mansfield
AU  - Nicholas Pytel
AU  - Christine M. Seroogy
AU  - Katelyn Watkin
AU  - Jo Wilson
AU  - Jonathan Wong
AU  - Therese Woodring
AU  - Katryn N. Furuya
PY  - 2026
DA  - 2026/11/03
JO  - JPGN reports
DO  - 10.1002/jpr3.70296
UR  - https://europepmc.org/article/PMC/PMC13729236
AB  - Patients presenting with hemophagocytic lymphohistiocytosis (HLH) are often critically ill, and rapid identification of both HLH and underlying triggers in secondary HLH is essential for successful treatment. We present a case of secondary HLH activated by disseminated histoplasmosis (DH) that mimicked autoimmune hepatitis (AIH) on liver histology and briefly led to an incorrect liver diagnosis. Once DH was identified, he was treated with antifungals in addition to HLH therapies and stabilized. He was subsequently found to have a heterozygous variant of uncertain significance in STAT1. Our case is the first in the literature to describe liver biopsies in a pediatric patient who has DH that had triggered HLH, and due to striking histology that mimicked AIH, important to report.
SN  - 2691-171X
ER  -