Favara B E, Steele A
Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Health, Hamilton, Montana, USA.
Pediatr Pathol Lab Med. 1997 Sep-Oct;17(5):769-87.
The morphology of Langerhans cell histiocytosis (LCH) involving lymph nodes was analyzed in 43 biopsies from 39 patients and findings were correlated with clinical data. Five histological motifs were recognized: sinusoidal, limited sinusoidal, epithelioid granulomatous, partial effacement, and total effacement. Lesions were composed of histiocytes of the Langerhans cell (LC) family, macrophages, multinucleated giant histiocytes, T lymphocytes, and eosinophils in varying proportions. Proliferative fractions ranged from 2.6 to 48% and 2 of 25 specimens showed a hyperdiploid aneuploid DNA ploidy profile. Epithelioid granulomas composed of histiocytes with the LC phenotype dominated three abdominal specimens, reflecting a picture of LCH not previously reported. Total effacement seen in three patients, was associated with unmarked histiocytoid cells, high proliferative fraction, aneuploid DNA ploidy profile, and, in two, a fatal outcome. Different histologic appearances in lesions from separate sites of the same patient were seen in the cases with epithelioid granulomas and in those with total effacement. The diverse histopathology in lymph nodes involved by LCH is considered in the context of current knowledge of this enigmatic disease.
对39例患者的43份淋巴结活检标本进行分析，以研究累及淋巴结的朗格汉斯细胞组织细胞增多症（LCH）的形态学，并将结果与临床数据相关联。识别出五种组织学模式：窦状、局限性窦状、上皮样肉芽肿性、部分消失和完全消失。病变由不同比例的朗格汉斯细胞（LC）家族的组织细胞、巨噬细胞、多核巨组织细胞、T淋巴细胞和嗜酸性粒细胞组成。增殖分数范围为2.6%至48%，25份标本中有2份显示超二倍体非整倍体DNA倍性图谱。由具有LC表型的组织细胞组成的上皮样肉芽肿在三份腹部标本中占主导，反映出一种此前未报道过的LCH表现。在三名患者中观察到的完全消失，与未标记的组织细胞样细胞、高增殖分数、非整倍体DNA倍性图谱相关，其中两名患者预后不良。在具有上皮样肉芽肿和完全消失的病例中，同一患者不同部位病变出现了不同的组织学表现。结合目前对这种疑难疾病的认识，对LCH累及淋巴结的多样组织病理学进行了探讨。
