Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria.
Department of Paediatrics and Adolescent Medicine, St. Anna Children's Hospital, Medical University of Vienna, Vienna, Austria.
Pediatr Transplant. 2024 Dec;28(8):e14884. doi: 10.1111/petr.14884.
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?
We evaluated immunohistochemical identification of LC (CD1a, CD207) in the proposita and in 14 ACR patient samples as distinguishing between ACR and recurrent LCH.
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.
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.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的表皮抗原呈递细胞恶性疾病。其特征是树突状细胞（Langerhans 细胞，LC）浸润各种组织，这些细胞表达 CD1a 或 CD207（朗格汉斯蛋白），常导致器官功能障碍。一位患有 LCH 的患者因 LCH 相关胆道疾病而行肝移植（LT）。LT 后发生胆管病。问题出现了：在这位患者中，受损肝移植物胆管上皮中的 LC 是否表示急性细胞排斥反应（ACR）或复发性 LCH？
我们评估了 LC（CD1a、CD207）在患者和 14 例 ACR 患者样本中的免疫组织化学鉴定，以区分 ACR 和复发性 LCH。
在 15 例患者样本中，有 3 例（20%）两种抗体均未标记。在其余 12 例样本（80%）中，有 4 例（26.7%）——包括患者样本——胆管上皮和周围门脉组织中有两种抗原标记的细胞，2 例（13.3%）在一个区域或另一个区域有两种抗原标记的细胞，但不在两个区域都有，6 例（40%）在一个区域或另一个区域只有一种抗原标记的细胞。
在没有 LCH 怀疑的情况下，ACR 中 CD1a 和 CD207/朗格汉斯蛋白的免疫染色可识别受损胆管中的 LC。这种生物标志物配对证明不是 LCH 特异性的。我们的发现表明，仅存在这些细胞不足以在移植肝中识别复发性 LCH。
