儿童间变性大细胞淋巴瘤31例误诊分析

Misdiagnosis Analysis of 31 Cases with Anaplastic Large Cell Lymphoma in Children

目的提高临床医师对儿童间变性大细胞淋巴瘤(ALCL)的认识,减少ALCL的早期误诊。方法对本院在2002年9月-2008年1月收治的曾误诊为其他疾病、入院后经病理检查确诊为ALCL的31例患儿的首发症状、临床表现及误诊原因进行回顾性分析。结果ALCL误诊率高达91.2%。误诊原因:1.ALCL早期即可发生结外侵犯,可累及各种组织和器官,首发症状多种多样,涉及多个专业,易造成误诊。2.肿瘤细胞可分泌IL-6、IL-9、IL-4、γ干扰素及巨噬细胞粒细胞集落刺激因子等多种细胞因子,使患儿出现发热,白细胞升高等类似炎性反应的表现,易与感染性疾病相混淆。3.ALCL的组织细胞形态学变异使病理学检查易发生误诊。4.临床医师和病理科医师对本病的临床和组织学特点均缺乏足够的认识。5.盲目使用激素,掩盖病情,影响病理结果。结论临床医师应对本病临床表现的多样化提高警惕,仔细查体,对治疗效果欠佳,同时侵犯多组淋巴结或有多处结外侵犯的患儿积极进行活检。明确诊断前不要盲目使用激素。病理科医师应对本病细胞形态学的多样性加强认识,正确选择免疫组织化学标志。作为诊断的唯一依据,其病理结果应由两家以上权威医院会诊证实。

Objective To make clinical doctors better understanding of anaplastic large cell lymphoma（ALCL） and reduce the misdiagnosis of ALCL at an earlier stage.Methods Retrospective analysis of clinical features in 31 children with ALCL from Sep.2002 to Jan.2008,who had been misdiagnosed as other diseases at first and latterly been confirmed as ALCL by pathological study.The reasons for mis-diagnosis and the symptoms of the disease were analyzed and reviewed.Results ALCL clinical misdiagnosis rate reached to 91.2%.The reasons for misdiagnosis were：1.The fact that extra-nodal involvement present in earlier stage,which caused ALCL to have diversified clinical symptoms,and the initial symptoms were diverse.2.ALCL cells produce multiple cytokines such as IL-6,IL-9,IL-4,and others.They might make patients have symptoms like inflammation.3.The morphology of ALCL was quite variable.4.The clinical doctors and pathologists did not have frofound understandings of ALCL.5.Inapropriate usage of steroid before diagnosis was mode.Conclusions Clinical doctors should be aware of the diversity of ALCL clinical symptoms and use steroid carefully,while pathologists should pay attention to morphological varieties of ALCL and choose appropriate immunohistiochemical stain markers to avoid misdiagnose.Pathologic diagnosis should be made by more than 2 oncology centers.