Fernández Miranda C, Martínez González M A, Pulido F, Rubio R, Asso A, González Mediero I, del Palacio A
Med Clin (Barc). 1989 Oct 21;93(12):449-56.
Forty cases of histiocytosis of Langerhans' cells (26 children and 14 adults) were evaluated; 20 were localized forms (with single or multiple bone involvement); another 20 were generalized forms, with organic dysfunction (lung, liver, hemopoietic system) in 7 children. This feature has a poor prognostic significance, as shown by the fact that 3 of these 7 patients died. In 3 patients single skin involvement was found, with spontaneous recovery one month after the diagnosis in one; in one case the single involvement was lymphadenopathic, associated to Hodgkin's lymphoma, and in another it was hypothalamic. These cases of single involvement have been followed up between 1 and 4 years. In 4 of 7 patients with generalized disease a reduction of OKT8 with an increased OKT4: OKT8 ratio was found, as reported in other cases from the literature. This finding gives support to an immunological rather than neoplastic basis for the disease. In all 40 cases the diagnosis was morphological, with positive staining for S-100 protein in 38.
对40例朗格汉斯细胞组织细胞增多症患者（26例儿童和14例成人）进行了评估；20例为局限性形式（单处或多处骨骼受累）；另外20例为全身性形式，7例儿童出现器官功能障碍（肺、肝、造血系统）。这一特征具有不良的预后意义，这7例患者中有3例死亡就证明了这一点。3例患者出现单处皮肤受累，其中1例在诊断后1个月自发恢复；1例单处受累为淋巴结病，与霍奇金淋巴瘤相关，另1例为下丘脑受累。这些单处受累病例已随访1至4年。在7例全身性疾病患者中的4例，发现OKT8降低，OKT4:OKT8比值升高，正如文献中其他病例所报道的那样。这一发现支持了该病的免疫学而非肿瘤学基础。在所有40例病例中，诊断均基于形态学，38例S-100蛋白染色呈阳性。
