Yu R C, Chu A C
Unit of Dermatology, Royal Postgraduate Medical School, London, U.K.
Br J Dermatol. 1996 Jul;135(1):36-41.
We have examined the clinicopathological correlates of 74 patients with histologically confirmed Langerhans cell histiocytosis. Factors that influenced disease outcome included, three or more organ/systems being involved, a disease onset before the age of 2 years, the involvement of certain vital organs/systems such as liver/spleen, bone marrow and lungs, and male gender. The total number of involved organs/systems was the single most important determinant of disease outcome. Mortality rate in patients with three or more organs/systems involved, was 26%, as compared with 0% in the group with one or two organs/systems involved (chi 2 = 11.2, P = 0.008). There were no familial cases in our series, but we looked for a possible immunogenetic association by tissue typing 46 Caucasian sufferers and comparing the results with 117 controls. We used normal peripheral blood lymphocytes in 39 cases, Epstein-Barr virus-transformed lymphoblastoid cell lines in 12 cases, and both peripheral blood and Epstein-Barr virus-transformed lymphocytes in five cases. The HLA-B7 antigen was significantly increased Langerhans cell histiocytosis patients (19 of 46 = 41.3%) compared with 19 of 117 (16.2%) in the control group chi 2 = 11.2, relative risk = 3.6, P value after correction = 0.013). Attempt to stratify the disease into single-system or multisystem disease did not result in any significant association.
我们研究了74例经组织学确诊的朗格汉斯细胞组织细胞增多症患者的临床病理相关性。影响疾病预后的因素包括：累及三个或更多器官/系统、2岁前发病、累及某些重要器官/系统（如肝脏/脾脏、骨髓和肺）以及男性。累及器官/系统的总数是疾病预后的唯一最重要决定因素。累及三个或更多器官/系统的患者死亡率为26%，而累及一个或两个器官/系统的患者组死亡率为0%（卡方=11.2，P=0.008）。我们的系列病例中没有家族性病例，但我们通过对46名白种人患者进行组织分型，并将结果与117名对照者进行比较，寻找可能的免疫遗传学关联。我们在39例中使用了正常外周血淋巴细胞，12例中使用了爱泼斯坦-巴尔病毒转化的淋巴母细胞系，5例中同时使用了外周血和爱泼斯坦-巴尔病毒转化的淋巴细胞。与对照组117名中的19名（16.2%）相比，朗格汉斯细胞组织细胞增多症患者中HLA - B7抗原显著增加（46名中的19名 = 41.3%）（卡方 = 11.2，相对风险 = 3.6，校正后P值 = 0.013）。将疾病分为单系统或多系统疾病的尝试未产生任何显著关联。
