Histiocytosis X: a review of the etiology, pathology, staging, and therapy.
Histiocytosis X: a review of the etiology, pathology, staging, and therapy.
复制标题
组织细胞增多症 X：病因学、病理学、分期和治疗的回顾。
DOI：
10.1002/mpo.2950140111
复制
发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Ellis,R
中科院分区：
文献类型：
--
作者：
Grundy,P;Ellis,R
第一个病人是一名2岁的白色女性，她于1984年7月因复发性右侧中耳炎1个月而住进费城儿童医院（CHP）。在第二次发作期间，在外耳道中注意到红色分泌物，并且在受影响的右耳后面出现灰色肿块。肿块逐渐增大并变为紫色。父母还指出，她最近有一个短暂的跛行涉及左髋关节。体格检查发现一个外观良好的孩子，右耳后有一个1.0 × 0.5厘米的坚硬皮下肿块，伴有潜在的身体缺陷。耳道被暗红色分泌物所掩盖。虽然步态正常，但左髋外展有轻微限制。平片显示右侧乳突、颞骨和岩骨、左侧耻骨支和右侧第三肋骨的穿孔性溶解性病变。血细胞计数、肝酶和尿分析均正常。乳突病变进行了活检，但完全刮除术被推迟，因为面神经不能很好地识别。光学显微镜检查显示嗜酸性粒细胞、淋巴细胞和多核巨细胞的组织细胞浸润，与组织细胞增多症X（HX）一致。电子显微镜证实存在朗格汉斯细胞颗粒。辐射，剂量为600拉德，分为四个150拉德的部分，仅针对颅骨病变，以阻止该过程，从而防止对面部神经和听觉器官的损伤。2个月后拍摄的X线片显示早期硬化，所有三个区域（治疗和未治疗）的愈合程度相同。
The first patient is a 2-year-old white female who was admitted to the Children’s Hospital of Philadelphia (CHP) in July 1984 with a 1-month history of recurrent, rightsided otitis media. During the second episode, a reddish discharge was noted in the external ear canal, and a greyish lump appeared behind the affected right ear. The lump enlarged progressively and became purple. The parents also noted that she had recently had a transient limp involving the left hip. Physical examination revealed a well-looking child with a 1.0-X-0.5-cm firm, subcutaneous mass behind the right ear with an associated underlying body defect. The ear canal was obscured by a dark red discharge. There was slight limitation of abduction of the left hip, although the gait was normal. Plain roentgenograms showed punched-out, lytic lesions of the right mastoid, temporal, and petrous bones, the left pubic ramus, and the right lateral third rib. Blood counts, liver enzymes, and urinalysis were normal. Biopsy of the mastoid lesion was performed, but complete curettage was deferred because the facial nerve could not be well identified. Light microscopy showed a histiocytic infiltrate with eosinophils, lymphocytes, and multinucleated giant cells, consistent with histiocytosis X (HX). Electron microscopy confirmed the presence of Langerhans cell granules. Radiation, a dose of 600 rad in four 150-rad fractions, was directed to the cranial lesion only, to arrest the process and thus prevent damage to the facial nerve and auditory apparatus. Roentgenograms taken 2 months later showed early sclerosis and healing of all three areas (treated and untreated) to an equal degree.