伴肺累及的新生儿朗格汉斯细胞组织细胞增生症1例

Neonatal Multisystem Langerhans Cell Histiocytosis with Pulmonary Involvement:A Case Report

患儿男,出生6 h,出生后即发现双臂孤立的溃疡性皮疹。皮损组织病理示:瘤细胞呈片、巢状分布,胞质丰富、淡染,细胞核呈肾形、咖啡豆样、有核沟,背景伴有组织细胞和嗜酸性粒细胞,真皮浅层片状肿瘤性朗格汉斯细胞浸润、嗜表皮性,符合朗格汉斯细胞组织细胞增生症改变;免疫组织化学染色示:S100(+)、CD1a(+)及CD207(+);胸部CT显示:双肺磨玻璃样改变,支气管血管束增粗,以右肺为著。纵膈及双侧腋窝淋巴结略增大。诊断为皮肤和肺均受累的多系统朗格汉斯细胞组织细胞增生症(multipy system-Langerhans cell histiocytosis,MS-LCH),予联合化疗,患儿痊愈。

A 6-hour-old male neonate presented with isolated ulcerative rash on his both arms after birth.Histopathology showed the tumor cells were distributed in sheets and nests,with abundant pale staining cytoplasm and kidney-shaped,coffee-bean-like nuclei with nuclear grooves.Histiocytes and eosinophils were present in the background,and the neoplastic Langerhans cells in the superficial dermis were invasive and epidermophilic.These changes were consistent with Langerhans cell histiocytosis.Immunochmistry showed S100(+),CD1a(+) and CD207(+).Chest CT found ground-glass opacities in both lungs,the bronchovascular bundle thickening especially in the right lung,and slightly enlarged mediastinal and bilateral axillary lymph nodes.The diagnosis of Multisystem-Langerhans cell histiocytosis(MS-LCH) involving skin and lung was established and the child recovered after the combination chemotherapy was employed.