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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。
Objective: To investigate the clinicopathological features and gene diagnosis of primary cutaneous anaplastic large cell lymphoma (C-ALCL). Methods: The clinical manifestations, histopathology and immunohistochemical staining of 6 cases of C-ALCL were observed. The T cell γ receptor (TCRγ) and heavy chain immunoglobulin (IgH) gene rearrangements Detection. Results: The clinical onset of solitary nodules more common, slow progression of the disease, some can subside on their own. Five patients were stable in treatment, one died of lymph node metastasis and liver metastasis. Microscopically, more than 75% of CD30 + interstitial macrophages were infiltrated with dermal and subcutaneous adipose tissue. Most of the tumor cells expressed T cell immunophenotype. TCRγ gene rearrangement was positive in 5 cases. Conclusion: C-ALCL is a rare primary malignant T-cell lymphoma with good prognosis. Comprehensive clinical manifestations, histopathological changes, immunohistochemistry and gene rearrangement test contribute to the correct diagnosis of the disease.