朗格汉斯细胞组织细胞增生症免疫表型及鉴别诊断

Immunophenotype and differential diagnosis of Langerhans cell histiocytosis

目的 观察朗格汉斯细胞组织细胞增生症（LCH）的组织形态学和免疫表型，以提高对此病变的诊断和鉴别诊断能力。方法 复习曾病理诊断为“嗜酸性肉芽肿”（包括诊断为组织细胞增生X和Hand-Schuller-Christian病）病例，按WHO（2001）造血与淋巴组织肿瘤分类中描述的组织形态学标准观察HE切片。并对病例进行随访。用单克隆抗体CD1a、S100、CD68、CD21、CD35、CD31、CD34、CD20、CD3、CD117、CD15、CD30、AE1／AE3、bcl-2、p53和ki-67进行免疫组化染色。结果 19例“嗜酸性肉芽肿”中有11例被证实为LCH，3例为淋巴结Kimura病，2例为淋巴瘤，3例为慢性炎症。误诊率高达42．1％。11例LCH中男女之比为4．5：1（1～22岁），平均年龄10岁；9例为骨单发病变，其中颅骨3例、长骨3例、短骨1例、其他扁骨2例；另1例为骨多发性病变（Hand—Schuller-Christian病）；1例为皮肤病变。所有病例的肿瘤CD1a和S-100均（＋），不同程度表达CD68；6例CD31弱（＋），1例AE1／AE3（＋），4例表达Ki-67（＋）（阳性率5％-10％），2例p53弱（＋），其余抗体染色均（-）。随访8例均存活。结论 诊断LCH的关键是根据具有表达CD1a和S-100的lanserhans细胞，部分病例可表达CD31，个别病例表达AE1／AE3。病理诊断应与霍奇金淋巴瘤、间变性大细胞淋巴瘤、Kimura病、血管淋巴细胞增生伴嗜酸细胞增多、恶性黑色素瘤和炎症进行鉴别。

Objective To observe the morphology and immtmophenotype of Langerhans cell histiocytosis （LCH） in order to increase the ability of diagnosis and differential diagnosis. Methods The HE stained sections of 19 cases, which had been diagnosed as eosinophilic granuloma including histiocytosis X and Hand-Schuller-Christian disease, were observed according to the morphologic features of IEH in WHO classification of tumor （2001）. Immunohistochemical staining of CD1a, S100, CD68, CD21, CD35, CD31, CD34, CD20, CD3, CD117, CD15, CD30, AE1/AE3, bcl-2, p53 and ki-67 was performed and analyzed in those diseases. Results In 19 eases of so-called ＂eosinophilie granuloma＂, 11 cases were confirmed as LCH, 3 as Kimura disease of lymph node, 2 as lymphoma and 3 as chronic inflammation. The rate of misdiagnosis was as high as 42.1%. The ratio of male to female in the cases of LCH was 4.5 ： 1, with age ranging 1-22 years （ average 10）. Nine cases occured solitarily, which included 3 in skulls, 3 in long bones, 1 in short bone and 2 in other flat bones; one case showed multifocal lesions but without system disease （Hand-Schidler-（Zristian disease）, and another case was involved in skin. All the cases expressed CD1a （9/9） and S-100 （11/11） obviously and CD68 （10/10） more or less; 6/9 cases were weakly positive for CD31, 1/9 cases had AE1/AE3- expression, 4/9 cases expressed ki-67 （positive rate was 5%-10%）, and 2/9 expressed 1953 weakly; the results of other antibodies were negative. Eight cases with follow-up （ 15-264 months） were all alive. Conclusions The pivotal basis of LCH diagnosis is the presence of Langerhans ceils with expression of CDla and S-100, CD31 in some cases, and AE1/AE3 in individual case. The LCH should be distinguished from Hodgkin lymphoma, anaplastic large cell lymphoma, Kimura disease, angiolymphoid hyperplasia with eosinophilia, malignant melanoma and chronic inflammation.