郎格汉斯细胞组织细胞增生症38例

Thirty-Eight Children with Langerhans Cell Histiocytosis

目的探讨儿童郎格汉斯细胞组织细胞增生症(LCH)的临床病理特点。方法收集皮肤科及儿内科1991年1月-2006年6月38例门诊及住院患儿资料,分析其临床、病理及免疫组织化学特点。结果平均发病年龄2.18岁,男女发病比例1.7:1。皮肤累及率78.9%,皮疹为首发症状达68.5%。皮疹主要分布于躯干,90%为出血性斑丘疹,3.3%呈脐凹样痂,类似急性痘疮样苔癣样糠疹。发热、肝脾大发生率分别为60.5%、68.4%、55.3%。淋巴结累及率31.6%,以颈部和腹股沟淋巴结为主。骨骼受损率31.6%,91.7%为单灶性,8.3%为多灶性损害。主要累及颅骨,还可累及腰椎、肱骨、髋骨等。呼吸道、外耳道、黏膜等累及率均不到10%。实验室检查贫血81.3%,T细胞亚群异常60.7%;EBV-IgG阳性32.1%。皮肤病理活组织检查26/30例显示真皮浅层较致密组织细胞浸润,伴淋巴细胞及少量嗜酸性粒细胞浸润。组织细胞胞质嗜酸性,核大呈肾形或咖啡豆样。4/30例浸润的组织细胞有明显的泡沫状胞质,嗜酸性粒细胞增多,偶见多核巨细胞。20/30例皮损免疫组织化学显示CD1a(+),S-100(+),KP-1(-)。4例淋巴结及4例颅骨肿块组织病理及免疫组织化学结果提示嗜酸性肉芽肿。结论LCH可累及多个脏器,表现多样,但皮疹尤其是出血性斑丘疹及发热、肝脾大较常出现。病理检查组织细胞核的形态有特异性,明确诊断需CD1a(+)、S100(+)。

Objective To explore the clinical and pathologic features of Langerhans cell histiocytosis （LCH）. Method Thirty - eight cases of LCH from both in hospital and outpatient department in dermatology and pediatry department from Jan. 1991 to Jun. 2006 were analyzed about the features of clinic ,pathology and immunohistochemistry. Results The mean age of onset was 2.18 years old. The male/female ratio was 1.7 ： 1. Skin lesions occurred in 78.9% of the patients. Among them,68.5% were as the first manifestation. The eruptions mainly distributed on trunk,90% of them presented as hemorrhagic maculopapules, nevertheless,3.3 % of the eruptions showed as crust with hilar depression,which was similar to pityriasis lichenoides etvarioliformis acuta. Fever, hepatomegalia and splenomegia occurred in patients at a rate of 60.5% ,68.4% ,55.3% respectively. Thirty- one point six percent of the patients had got lymphadenectasis ,the neck and inguinal lymph nodes were the common site to be affected. Ossature involvement occurred in 31.6% of the patients ,which 8.3% got multiple injuries, howevet,91.7% got a solitary bone involved. Skull was the main site to be injured, else were lumbar, humerus, hipbone, an so on. Respiratory tract, auditory canal, mucosa were also the sites involved in this disease, but the incidence rate was lower than 10% , respectively. The laboratory data showed that 81.3% of the patients were anaemia ,60.7% with abnormal subgroup of T - cell, and 32.1% positive for EBV - IgG. The skin histopathology data of 26/30 cases revealed that lichenoid infiltrates of Langerhans cells confined to the upper dermis. Cytologic features were cells with abundant eosinophilic cytoplasms, and longitudinally grooved or reniform nuclei. Lymphocytes and a few eosinophils also could be seen. Four cases of thirty showed that the proliferative Langerhans cells were with pale cytoplasms, besides, there were numerous eosinophils, and sometimes a few multinucleate cells were scattered. The immunity test of 20 cases of thirty displayed that CD1 a（ ＋ ）S100 （ ＋ ）KP - 1 （ - ）. Biopsy of lymphaden and tumor of the skull of the rest 8 patients were all diagnosed as eosinophilic granuloma through both hematoxylin and eosin - stained section and immune marks. Conclusions Multiple systems can be involved in LCH. Hemorrhagic maculopapules, fever and splenohepatomegalia are common presentations in this disease. The morphous of nucles of histiocytes is particular, and to diagnose definitely,both CD1a（ ＋ ） and S-100（ ＋ ） are needed.