Abellán Martínez M C, Méndez Martínez P, Sánchez Gascón F, Hernández Martínez J, Sánchez Montón T, Romero Mas E
Servicio de Neumología, Hospital General Universitario de Murcia.
An Med Interna. 2002 Jan;19(1):16-8.
Histiocytosis X or Langerhans cell histiocytosis is an infrequent disease, which consists on proliferation of Langerhans cells. The etiology is unknown. Diagnosis is reached by electron microscope study of the biopsy, in which the Birbeck intracytoplasmatic granules of the Langerhans cells are found and/or by immunohistochemistry procedures able to detect S-100 antigen and CD1 cells. Diagnosis can also be reached with a bronchoalveolar lavage in which CD1 cells will appear in a score higher than 5%. We present the case of a 16 year-old girl that first appeared with a pulmonary lesion with a honey comb X-ray pattern and unsyntomatic mandibular bone affectation. Diagnosis was reached by biopsy study using S-100 antigen detection procedures. We consider this case an important one due to the infrequency of this particular disease.
组织细胞增多症X或朗格汉斯细胞组织细胞增多症是一种罕见疾病，由朗格汉斯细胞增殖构成。病因不明。通过对活检组织进行电子显微镜研究来确诊，在该研究中可发现朗格汉斯细胞的伯贝克胞质内颗粒，和/或通过能够检测S-100抗原和CD1细胞的免疫组织化学程序来确诊。也可通过支气管肺泡灌洗来确诊，其中CD1细胞出现的比例会高于5%。我们报告一例16岁女孩的病例，该女孩最初表现为肺部病变，X线呈蜂窝状，下颌骨无症状受累。通过使用S-100抗原检测程序的活检研究确诊。鉴于这种特殊疾病的罕见性，我们认为该病例很重要。
