Chen B F, Liang D C
Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1994 Aug;54(2):116-21.
Langerhans cell histiocytosis encompasses a wide clinical spectrum, from a benign localized disease to acute generalized disease with fatal outcome. Here the histological features and clinical data for thirteen patients are reviewed and compared. The antigenic phenotype of the proliferating cells in Langerhans cell histiocytosis was also studied.
The antibodies used included S-100 protein, vimentin, peanut agglutinin, HLA-DR, leukocyte common antigen, UCHL-1, CD 20, lysozyme, alpha 1-antitrypsin and epithelial membrane antigen.
Six patients had localized lesions affecting a bone or its surrounding soft tissue. Four patients had multifocal disease without organ dysfunction. All patients of these two categories survived, except for one adult who died of hypertensive cerebral hemorrhage. Three patients had multifocal disease with liver, lung or bone marrow dysfunction. One died and two were lost to follow up.
There was no significant difference in pathological findings between localized disease and multifocal disease. Immunohistochemical examinations using a panel of antibodies, including S-100 protein, peanut agglutinin, HLA-DR and vimentin, were very helpful for diagnosis of Langerhans cell histiocytosis.
朗格汉斯细胞组织细胞增多症涵盖广泛的临床谱，从良性局限性疾病到具有致命结局的急性全身性疾病。本文对13例患者的组织学特征和临床资料进行回顾并比较。同时还研究了朗格汉斯细胞组织细胞增多症中增殖细胞的抗原表型。
所使用的抗体包括S-100蛋白、波形蛋白、花生凝集素、HLA-DR、白细胞共同抗原、UCHL-1、CD20、溶菌酶、α1-抗胰蛋白酶和上皮膜抗原。
6例患者有累及骨骼或其周围软组织的局限性病变。4例患者有多灶性病变但无器官功能障碍。这两类患者中，除1例死于高血压脑出血的成人外，其余均存活。3例患者有多灶性病变伴肝、肺或骨髓功能障碍。1例死亡，2例失访。
局限性疾病和多灶性疾病的病理表现无显著差异。使用一组抗体（包括S-100蛋白、花生凝集素、HLA-DR和波形蛋白）进行免疫组化检查对朗格汉斯细胞组织细胞增多症的诊断非常有帮助。
