原发性皮肤CD30阳性T细胞淋巴增生性病变5例临床病理分析

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182、1、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(),4例CD45RO呈(),仅1例CD3呈(),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。

Primary cutaneous CD30-positive T-cell lymphoproliferative disorders: a clinicopathological analysis of 5 cases

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 multiple 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.