Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Dec;28(8):e14884.
doi: 10.1111/petr.14884.

Langerhans Cell Histiocytosis or Acute Cellular Rejection?

Andreas Entenmann  1 , Hubert Kogler  2 , Wolf-Dietrich Huber  3 , Marita Kölz  4 , A S Knisely  5 , Kristijan Skok  5
Affiliations

Langerhans Cell Histiocytosis or Acute Cellular Rejection?

Andreas Entenmann et al. Pediatr Transplant. 2024 Dec.

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare malignant disorder of epidermal antigen presenting cells. It is characterized by infiltration of various tissues with dendritic cells (Langerhans cells, LC) that express CD1a or CD207 (langerin), often leading to organ dysfunction. A patient with LCH required liver transplantation (LT) for LCH-associated biliary-tract disease. Cholangiopathy developed after LT. The question arose: In this patient, did LC in damaged liver-allograft biliary epithelium signify acute cellular rejection (ACR) or recurrent LCH?

Methods: We evaluated immunohistochemical identification of LC (CD1a, CD207) in the proposita and in 14 ACR patient samples as distinguishing between ACR and recurrent LCH.

Results: Among 15 patient samples, 3 (20%) marked with neither antibody. Among the remaining 12 samples (80%), 4 (26.7%)-including that from the proposita-had cells marking for both antigens within bile-duct epithelium as well as in surrounding portal-tract connective tissue, 2 (13.3%) had cells marking for both antigens in one region or the other, but not in both, and 6 (40%) had cells marking for only one antigen in one region or the other.

Conclusions: Immunostaining for CD1a and CD207/langerin in the setting of ACR without suspicion of LCH identifies LC in damaged bile ducts. This biomarker pairing proved not to be LCH-specific. Our findings indicate that the presence of these cells alone is insufficient to identify recurrent LCH in the allograft liver.

Keywords: CD1a; Langerhans cell histiocytosis; acute cellular rejection; langerin; liver.

PubMed Disclaimer

References

    1. K. Chikwava and R. Jaffe, “Langerin (CD207) Staining in Normal Pediatric Tissues, Reactive Lymph Nodes, and Childhood Histiocytic Disorders,” Pediatric and Developmental Pathology 7, no. 6 (2004): 607–614, https://doi.org/10.1007/s10024‐004‐3027‐z.
    1. J. F. Emile, O. Abla, S. Fraitag, et al., “Revised Classification of Histiocytoses and Neoplasms of the Macrophage‐Dendritic Cell Lineages,” Blood 127, no. 22 (2016): 2672–2681, https://doi.org/10.1182/blood‐2016‐01‐690636.
    1. G. Goyal, A. Tazi, R. S. Go, et al., “International Expert Consensus Recommendations for the Diagnosis and Treatment of Langerhans Cell Histiocytosis in Adults,” Blood 139, no. 17 (2022): 2601–2621, https://doi.org/10.1182/blood.2021014343.
    1. J. Menon, A. Rammohan, M. Vij, N. Shanmugam, and M. Rela, “Current Perspectives on the Role of Liver Transplantation for Langerhans Cell Histiocytosis: A Narrative Review,” World Journal of Gastroenterology 28, no. 30 (2022): 4044–4052, https://doi.org/10.3748/wjg.v28.i30.4044.
    1. S. Feng, L. Han, M. Yue, et al., “Frequency Detection of BRAF V600E Mutation in a Cohort of Pediatric Langerhans Cell Histiocytosis Patients by Next‐Generation Sequencing,” Orphanet Journal of Rare Diseases 16, no. 1 (2021): 272, https://doi.org/10.1186/s13023‐021‐01912‐3.

LinkOut - more resources