以呼吸系统症状为首发表现的间变性大细胞淋巴瘤一例及文献复习

Anaplastic large cell lymphoma in which pulmonary symptoms were first presented： a case report with literature review

目的 提高对以呼吸系统症状为首发表现的间变性大细胞淋巴瘤（ALCL）的临床、实验室检查、胸部影像学和病理改变的认识。方法 对1例经纤维支气管镜肺活检和骨髓活检证实的ALCL病例的临床表现、实验室检查、影像学和病理资料并结合相关文献进行回顾性分析。结果 以呼吸系统症状为首发表现的ALCL症状主要表现为咳嗽、胸痛、咳血痰、气促、发热；实验室检查可有三系血细胞减少、脾大、骨髓噬血细胞增多；胸部X线和高分辨率CT（HRCT）早期可表现双肺多个小点状结节影，后可出现肺不张、肺团块影和纵隔淋巴结肿大，晚期可出现大量胸腔积液。ALCL的病理表现为肿瘤细胞体积大，核大而不规则，细胞弥散或巢样沿淋巴窦或滤泡间浸润，几乎所有的瘤细胞均表达间变细胞CD30（Ki-1）抗原。结论 以呼吸系统症状为首发表现的ALCL非常罕见，其临床表现、影像学检查无特异性，而诊断需要依靠病理，CD30（ki-1）阳性是诊断ALCL的有力依据。

Objective To highlight the characteristics of anaplastic large cell lymphoma（ALCL） in which pulmonary symptoms were first presented. Methods Case report and review of the related literatures were summarized. The clinical symptoms, laboratory test, radiographic patterns,diagnosis and treatment of ALCL were described. Results A 49-year-old female patient presented cough, chest pain, haemoptysis, dyspnea,fever,pancytopenia,splenomegaly,and hemophagocytic syndrome（HPS）. Radiographically,bilateral nodus, pulmonary atelectasis, bolus in lung,mediastinal lymphadenectasis and pleural effusion could be seen. Large tumor cell and primary CD30/Ki-1 positive were presented in almost all pathological findings. Conclusions ALCL in which pulmonary symptoms were first presented is very rare. The clinical manifestations and radiography of this kind of ALCL are non-specific. Positive CD30/Ki-1 is an important indicator. Lymphoma must be alert if infective reason can not be found and unknown cells are presented in hemophagocytic syndrome cases.