肉芽肿性皮肤松弛症继发间变性淋巴瘤激酶阴性间变性大细胞淋巴瘤一例并文献复习

Granulomatous slack skin with ALK-negative secondary anaplastic large-cell lymphoma：report of one case and review of literature

目的：提高对肉芽肿性皮肤松弛症（GSS）继发间变性淋巴瘤激酶（ALK）阴性间变性大细胞淋巴瘤（ALK-ALCL）的诊断、鉴别诊断、治疗及预后的认识。方法报道1例GSS继发ALK-ALCL患者的诊治经过，并进行相关文献复习。结果该患者因皮肤斑块、左腋下包块活组织检查确诊为GSS，经激素、干扰素、硼替佐米联合化疗等治疗，病情迁延不愈。4年后出现干咳、发热，经支气管镜肺活组织检查确诊为ALK-ALCL，左腋下包块穿刺活组织检查确诊为GSS。给予CHOP方案化疗效果欠佳，换用GEMOX＋DEX方案治疗病情有所改善，终因肺部感染死亡。结论 GSS继发ALCL罕见，诊断依据病理学检查，治疗以化疗为主，预后主要取决于ALCL的治疗效果。

Objective To enhance the understanding of the diagnosis, differential diagnosis, treatment and prognosis of granulomatous slack skin （GSS） with secondary ALK-negative anaplastic large-cell lymphoma （ALK-ALCL）. Methods One case of GSS secondary ALK-ALCL was reported, and related literature was reviewed. Results The patient was diagnosed as GSS by biopsy of skin patches and left armpit mass. After treatment of cortical hormone,interferon and bortezomib in combination with chemotherapy, favorable efficacy could not gain. With the following occurrence of dry cough and fever after 4 years, the patient was diagnosed as ALK-ALCL by transbronchial lung biopsy, and GSS by biopsy of left armpit mass. The chemotherapy with CHOP regimen was performed, but was not effective, and then the patient＆#39;s condition was improved after the chemotherapy with GEMOX＋DEX. Eventually the patient died of pulmonary infection. Conclusions GSS with secondary ALCL is very rare, which is diagnosed with pathology. Chemotherapy is its main treatment, and the prognosis depends on the treatment effect of ALCL.