以皮损为首发表现的淋巴母细胞淋巴瘤/白血病六例临床病理分析

目的 探讨以皮损为首发表现的淋巴母细胞淋巴瘤/白血病的临床、组织病理表现及免疫组化特点.方法 分析中国医学科学院皮肤病医院2012-2015年诊断的6例以皮损为首发表现的淋巴母细胞淋巴瘤/白血病的临床和组织病理特点.结果 6例患者中男4例、女2例;儿童和青年4例,成人2例.中位发病年龄13.5岁,平均病程8.5个月.皮损表现为单发(1例)或多发(5例)结节或浸润性斑块,组织病理学表现为真皮及皮下脂肪内形态单一、中等大小、胞质较少、染色质细腻的淋巴样细胞增生,可见小核仁,无亲表皮现象,1例可见星空现象.2例免疫学表型符合B淋巴母细胞淋巴瘤,2例符合T淋巴母细胞淋巴瘤,2例呈现T、B双系表型.该病治疗困难,1例患儿在化疗后获缓解,2例患者在接受化疗后皮损部分改善.结论 皮损表现和组织学无法区别B和T淋巴母细胞淋巴瘤,只有通过免疫表型进行鉴别,早期骨髓及影像学检查尤为重要.

Clinicopathological analysis of six cases of lymphoblastic lymphoma/leukemia with skin lesions as the initial manifestation

Objective To investigate clinical manifestations,histopathological and immunohistochemical features of lymphoblastic lymphoma/leukemia with skin lesions as the initial manifestation.Methods Clinical data were collected from 6 cases of lymphoblastic lymphoma/leukemia initially manifesting as skin lesions in Hospital of Dermatology of Chinese Academy of Medical Sciences and Peking Union Medical College between 2012 and 2015,and clinical and histopathological features were retrospectively analyzed.Results The 6 patients included 4 males and 2 females,of whom,4 were children or youths,and 2 were adults.The median age of onset was 13.5 years,and the median disease duration was 8.5 months.The skin lesions mainly manifested as single (1 case) or multiple (5 cases) nodules or infiltrative plaques.Histopathological examination showed dense monomorphous infiltrates composed of atypical lymphoid cells in the dermis and subcutaneous adipose tissue.The tumor cells in most cases were mediumsized with scant cytoplasm,stippled chromatin and small nucleoli.The infiltration of lymphoid cells was separated from the epidermis by a grenz zone.A'starry sky'appearance was observed in 1 case.Based on the immunopheno-types,2 cases were diagnosed as B-cell lymphoblastic lymphoma,2 as T-cell lymphoblastic lymphoma,and other 2 as B/T-cell biphenotypic lymphoblastic lymphoma.The treatment of this disease was difficult.After chemotherapy,1 patient achieved complete remission,and 2 patients achieved partial remission.Conclusion B-cell and T-cell lymphoblastic lymphoma have similar clinical and histopathological characteristics,and can only be distinguished from each other based on immunophenotypes,thus early bone marrow and imaging examinations are particularly important.