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Gene rearrangement in T-cell receptor γ and immunoglobulin heavy chain for primary cutaneous anaplastic large cell lymphoma
Author(s): 
Pages: 3-6
Year: Issue:  1
Journal: JOURNAL OF CLINICAL DERMATOLOGY

Keyword:  淋巴瘤T细胞皮肤基因重排T细胞γ受体免疫球蛋白重链;
Abstract: 目的:探讨原发性皮肤间变性大细胞淋巴瘤(C-ALCL)临床病理特点和基因诊断方法.方法:对6例C-ALCL的临床表现、病理形态学和免疫组化染色进行观察,并用PCR方法对石蜡标本进行T细胞γ受体(TCRγ)和重链免疫球蛋白(IgH)基因重排检测.结果:临床起病以孤立性结节多见,病情进展缓慢,个别可自行消退.5例患者经治疗病情稳定,1例死于淋巴结及肝脏转移.镜下以75%以上CD30+间变性大细胞弥漫浸润真皮及皮下脂肪组织为特点,多数瘤细胞表达T细胞免疫表型.5例标本TCRγ基因重排阳性.结论:C-ALCL是少见的原发皮肤的低度恶性T细胞性淋巴瘤,预后较好.综合临床表现、组织病理改变、免疫组化及基因重排检测有助于本病的正确诊断.
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