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Entenmann, A; Kogler, H; Huber, WD; Kölz, M; Knisely, AS; Skok, K.
Langerhans Cell Histiocytosis or Acute Cellular Rejection?
Pediatr Transplant. 2024; 28(8): e14884 Doi: 10.1111/petr.14884 (- Case Report)
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Führende Autor*innen der Med Uni Graz
Skok Kristijan
Co-Autor*innen der Med Uni Graz
Knisely Alexander
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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.
Find related publications in this database (using NLM MeSH Indexing)
Humans - administration & dosage
Histiocytosis, Langerhans-Cell - diagnosis
Graft Rejection - immunology
Antigens, CD1 - metabolism
Liver Transplantation - administration & dosage
Male - administration & dosage
Lectins, C-Type - metabolism
Mannose-Binding Lectins - metabolism
Antigens, CD - metabolism
Female - administration & dosage
Immunohistochemistry - administration & dosage
Infant - administration & dosage
Child - administration & dosage
Diagnosis, Differential - administration & dosage
Liver - pathology
Child, Preschool - administration & dosage
Recurrence - administration & dosage
Langerhans Cells - administration & dosage

Find related publications in this database (Keywords)
acute cellular rejection
CD1a
Langerhans cell histiocytosis
langerin
liver
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