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机构地区:[1]北京大学第一医院病理科,北京100034 [2]北京大学第一医院皮肤科,北京100034
出 处:《诊断病理学杂志》2005年第6期419-422,i0011,共5页Chinese Journal of Diagnostic Pathology
摘 要:目的探讨原发性皮肤CD30阳性T细胞淋巴增生性病变的临床病理特征、诊断标准和预后.方法参照欧洲癌症研究和治疗组织及WHO分类标准对5例该病进行光镜观察、免疫组织化学标记和随访研究.结果5例中3例为淋巴瘤样丘疹病(LyP),均为女性,年龄分别为18、21、52岁;2例为原发性皮肤间变性大细胞淋巴瘤(C-ALCL),均为男性,年龄70和72岁;3例LyP临床表现为较广泛、反复发作和可自然消退的皮肤丘疹,2例C-ALCL则表现较为限局多发或单发的皮肤肿块;镜下为不同程度的炎性背景中有中~大型异型淋巴样细胞散在或弥漫浸润真皮.免疫组化标记5例异型细胞CD30均呈(卅),4例CD45RO呈(卅),仅1例CD3呈(卅),3例granzyme B呈(+~++),5例EMA、ALK、CD56均(-).2例C-ALCL中,1例于确诊后9个月死亡,另1例截趾;3例LyP缓解,维持药量治疗,病情稳定.结论 C-ALCL和LyP在临床表现和组织学上多有重叠,但临床经过、治疗及预后不同,在确定诊断和选择治疗时,临床表现及其经过是最重要的依据.Objective To investigate the clinicopathological features, diagnostic criteria and prognosis of primary cutaneous CD30-positive T-cell lymphoproliferative disorders. Methods 5 cases were studied by light microscopy and immunohistochemistry. Results Among 5 cases, 3 were lymphomatoid papulosis (LyP), all were female patients, 18, 21, 52 years old respectively. 2 were primary cutaneous anaplastic large-cell lymphomas (C-ALCL), both were male, 70 and 72 years old. Clinically,the 3 LyP presented muhiple recurrent, self-healing skin papules and the 2 C-ALCL showed localized masses, Histologically, the 5 cases showed various numbers of large atypical lymphoid cells infiltrated in a various inflammation background. Immunohistochemically, the atypical lymphoid cells of 5 cases manifested strong CD30 reaction; the strong immunostaining was also found in 4 cases for CD45RO (4/4), 1 for CD3 (1/5) ; intermediate and weak positive reaction was found in 3 cases for granzyme B staining; all 5 cases showed negative reaction for CD56, EMA and ALK. Follow-up showed that 1 C- ALCL died after 9 months of diagnosis, the another had toe amputation, while 3 cases of LyP was in a stable condition. Conclusion C-ALCL and LyP reveal some overlapping clinical and histologic features, but their clinical course and prognosis are different. The correct diagnosis requires assessment of clinical appearance, histological and phenotypic features.
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