Gao Kehai, Li Hongtao, Huang Caihong, Li Huazhuang, Fang Jun, Tian Chen
Department of Orthopaedics, Yidu Central Hospital, Shandong, People's Republic of China.
Onco Targets Ther. 2016 Jul 1;9:3993-6. doi: 10.2147/OTT.S109746. eCollection 2016.
Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma that has strong expression of CD30. ALCL can sometimes involve the bone marrow, and in advanced stages, it can produce destructive extranodal lesions. But anaplastic large cell lymphoma kinase (ALK)+ ALCL with soft tissue involvement is very rare.
A 35-year-old woman presented with waist pain for over 1 month. The biopsy of soft tissue lesions showed that these cells were positive for ALK-1, CD30, TIA-1, GranzymeB, CD4, CD8, and Ki67 (90%+) and negative for CD3, CD5, CD20, CD10, cytokeratin (CK), TdT, HMB-45, epithelial membrane antigen (EMA), and pan-CK, which identified ALCL. After six cycles of Hyper-CVAD/MA regimen, she achieved partial remission. Three months later, she died due to disease progression.
This case illustrates the unusual presentation of ALCL in soft tissue with a bad response to chemotherapy. Because of the tendency for rapid progression, ALCL in young adults with extra-nodal lesions are often treated with high-grade chemotherapy, such as Hyper-CVAD/MA.
间变性大细胞淋巴瘤(ALCL)是一种非霍奇金淋巴瘤,其CD30表达强烈。ALCL有时可累及骨髓,在晚期可产生破坏性结外病变。但伴有软组织受累的间变性大细胞淋巴瘤激酶(ALK)阳性ALCL非常罕见。
一名35岁女性出现腰背痛1个多月。软组织病变活检显示这些细胞ALK-1、CD30、TIA-1、颗粒酶B、CD4、CD8和Ki67(90%以上)呈阳性,而CD3、CD5、CD20、CD10、细胞角蛋白(CK)、末端脱氧核苷酸转移酶(TdT)、HMB-45、上皮膜抗原(EMA)和全细胞角蛋白呈阴性,确诊为ALCL。经过六个周期的Hyper-CVAD/MA方案治疗后,她达到部分缓解。三个月后,她因疾病进展死亡。
该病例说明了ALCL在软组织中的不寻常表现以及对化疗反应不佳。由于其快速进展的趋势,伴有结外病变的年轻成人ALCL通常采用高强度化疗,如Hyper-CVAD/MA。