Clavio M, Rossi E, Truini M, Carrara P, Ravetti J L, Spriano M, Vimercati A R, Santini G, Canepa L, Pierri I, Celesti L, Miglino M, Castellaneta A, Damasio E, Gobbi M
Department of Internal Medicine (DIMI), University of Genox, Italy.
Leuk Lymphoma. 1996 Jul;22(3-4):319-27. doi: 10.3109/10428199609051763.
Fifty-three consecutive cases of adult CD30+ anaplastic large cell lymphoma (ALCL) have been analyzed. Thirty-six were classified as Hodgkin's disease like variety (HL) (67%) and seventeen as so-called common type (CT) (33%). All cases strongly expressed the CD30/Ki-1 antigen; the neoplastic cells expressed CD15, CD45 and EMA in 60%, 44% and 33% of cases, respectively; T. B and null phenotypes were found in 37%, 17% and 46% of cases. Bulky mediastinal, B symptoms, and extranodal disease at diagnosis were present in 36%, 49% and 25% of cases. EBV encoded latent membrane protein (LMP-1) was found in 10 cases. Of the 13 tested cases only 4 expressed a weak positivity of the CD40 molecule, in a fraction of the tumor cells; in the same cases CD21 was never found. Patients were treated with various protocols; of the 50 evaluable patients, 39 (78%) obtained a complete remission (CR), 3 (6%) a partial remission (PR) and 8 (16%) did not respond. The projected overall disease free survival (DFS) at 36 months is 70%. Only patients with advanced disease stage (III-IV) showed a statistically decreased DFS and survival. Only symptomatic and extranodal disease significantly appeared to influence survival. This study confirms the good outcome of this group of lymphomas and differs from other reports for some clinical (lower percentage of advanced stage, extranodal disease and skin infiltration) and pathological (HL/CT ratio and immunophenotype) features.
对53例连续性成人CD30 + 间变性大细胞淋巴瘤（ALCL）病例进行了分析。36例被归类为霍奇金病样型（HL）（67%），17例为所谓的普通型（CT）（33%）。所有病例均强烈表达CD30/Ki-1抗原；肿瘤细胞分别在60%、44%和33%的病例中表达CD15、CD45和EMA；T、B和无表型分别在37%、17%和46%的病例中发现。诊断时，36%的病例有巨大纵隔、49%有B症状、25%有结外病变。10例病例中发现EBV编码的潜伏膜蛋白（LMP-1）。在13例检测病例中，只有4例在部分肿瘤细胞中表达CD40分子弱阳性；在相同病例中从未发现CD21。患者接受了各种方案治疗；在50例可评估患者中，39例（78%）获得完全缓解（CR），3例（6%）获得部分缓解（PR），8例（16%）无反应。预计36个月时的总体无病生存率（DFS）为70%。只有晚期疾病阶段（III-IV期）的患者DFS和生存率有统计学意义的下降。只有有症状和结外病变似乎显著影响生存率。本研究证实了这组淋巴瘤的良好预后，并且在一些临床（晚期、结外病变和皮肤浸润的较低百分比）和病理（HL/CT比例和免疫表型）特征方面与其他报告不同。
