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Peer-reviewedCC BY-NC-ND 4.0ACG Case Reports Journal

Hepatic Sarcoidosis as a Rare Cause of Secondary Hemophagocytic Lymphohistiocytosis

  • Hamzah Shariff1
  • Smriti Nair1
  • Akash Patel2
  • Alberto Martinez Mencia3
  • Wei Jiang3
  • Julianne Qualtieri3
  • Adam Binder4
  • Fabian Mendoza5
  • David A. Sass2
5 affiliations
  1. 1Department of Internal Medicine, Thomas Jefferson University Hospital, Philadelphia, PA
  2. 2Division of Gastroenterology and Hepatology, Thomas Jefferson University Hospital, Philadelphia, PA
  3. 3Department of Pathology, Anatomy, and Cell Biology, Thomas Jefferson University Hospital, Philadelphia, PA
  4. 4Department of Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, PA
  5. 5Division of Rheumatology, Thomas Jefferson University Hospital, Philadelphia, PA
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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome often triggered by an underlying disease process. Macrophage activation syndrome (MAS) is a secondary HLH phenotype associated with autoimmune disease. A 72-year-old man presented with fever, pancytopenia, hyperferritinemia, splenomegaly, and acute liver injury. Bone marrow biopsy demonstrated hemophagocytosis, supporting HLH. Despite corticosteroid therapy, progressive liver injury prompted liver biopsy, which revealed sinusoidal hemophagocytosis and noncaseating granulomatous inflammation consistent with hepatic sarcoidosis. Infectious and malignant evaluations were unrevealing. This case highlights hepatic sarcoidosis as a rare underlying driver of secondary macrophage activation syndrome/HLH and the importance of tissue diagnosis in patients with severe hepatic involvement.

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Cite

BibTeXRIS
BibTeX
@article{shariff2026hepatic,
  title = {{Hepatic Sarcoidosis as a Rare Cause of Secondary Hemophagocytic Lymphohistiocytosis}},
  author = {Hamzah Shariff and Smriti Nair and Akash Patel and Alberto Martinez Mencia and Wei Jiang and Julianne Qualtieri and Adam Binder and Fabian Mendoza and David A. Sass},
  journal = {ACG Case Reports Journal},
  year = {2026},
  month = nov,
  doi = {10.14309/crj.0000000000002379},
  url = {https://doi.org/10.14309/crj.0000000000002379},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Hepatic Sarcoidosis as a Rare Cause of Secondary Hemophagocytic Lymphohistiocytosis
AU  - Hamzah Shariff
AU  - Smriti Nair
AU  - Akash Patel
AU  - Alberto Martinez Mencia
AU  - Wei Jiang
AU  - Julianne Qualtieri
AU  - Adam Binder
AU  - Fabian Mendoza
AU  - David A. Sass
PY  - 2026
DA  - 2026/11/04
JO  - ACG Case Reports Journal
DO  - 10.14309/crj.0000000000002379
UR  - https://doi.org/10.14309/crj.0000000000002379
AB  - Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome often triggered by an underlying disease process. Macrophage activation syndrome (MAS) is a secondary HLH phenotype associated with autoimmune disease. A 72-year-old man presented with fever, pancytopenia, hyperferritinemia, splenomegaly, and acute liver injury. Bone marrow biopsy demonstrated hemophagocytosis, supporting HLH. Despite corticosteroid therapy, progressive liver injury prompted liver biopsy, which revealed sinusoidal hemophagocytosis and noncaseating granulomatous inflammation consistent with hepatic sarcoidosis. Infectious and malignant evaluations were unrevealing. This case highlights hepatic sarcoidosis as a rare underlying driver of secondary macrophage activation syndrome/HLH and the importance of tissue diagnosis in patients with severe hepatic involvement.
SN  - 2326-3253
ER  -