Shibuya A, Muraki Y, Satoh Y, Kobayashi T, Kojima H, Nagasawa T, Abe T, Tomiyama J, Kudoh H, Adachi Y
Division of Hematology, University of Tsukuba.
Rinsho Ketsueki. 1992 Jun;33(6):770-5.
Clinicopathological analyses of 6 cases of mantle zone lymphoma (MZL) were carried out. The median age of the patients was 62 years with a range of 41 to 71 years and the male-to-female ratio was 1:1. Superficial lymph node (LN) swelling was present only in 2 patients. Giant LN swellings of the mesenteric or inguinal regions were present in 4, and bone marrow involvement by lymphoma cells in 5. Serum protein electrophoresis revealed a monoclonal protein of IgM kappa type in 2 patients. One of these also had polyclonal hypergammaglobulinemia. An immunohistochemical study of 6 patients revealed LN-1-(-)+, LN-2+(-)++, sIgM+, sIgD+, CALLA +/-(-)+, DRC-1+(-)++. The immunohistochemical features of the cases were similar to those of small lymphocytic lymphoma or follicular lymphoma. Only 1 patients out of 6 achieved complete remission. Two patients died, one of pneumonia after chemotherapy and the other of cancer. The others were alive 4 to 100 months after the diagnoses. Although giant LN swelling and bone marrow involvement of lymphoma cells which were refractory to treatments were frequently observed, we consider MZL to be a slowly progressive and low-grade type of non-Hodgkin's lymphoma.
对6例套区淋巴瘤（MZL）进行了临床病理分析。患者的中位年龄为62岁，范围为41至71岁，男女比例为1:1。仅2例患者出现浅表淋巴结肿大。4例出现肠系膜或腹股沟区巨大淋巴结肿大，5例出现淋巴瘤细胞骨髓受累。血清蛋白电泳显示2例患者存在IgM κ型单克隆蛋白。其中1例还伴有多克隆高丙种球蛋白血症。对6例患者的免疫组织化学研究显示LN-1-(-)+、LN-2+(-)++、sIgM+、sIgD+、CALLA +/-(-)+、DRC-1+(-)++。这些病例的免疫组织化学特征与小淋巴细胞淋巴瘤或滤泡性淋巴瘤相似。6例中仅1例实现完全缓解。2例死亡，1例化疗后死于肺炎，另1例死于癌症。其他患者在诊断后存活4至100个月。尽管经常观察到对治疗难治的巨大淋巴结肿大和淋巴瘤细胞骨髓受累，但我们认为MZL是一种进展缓慢的低级别非霍奇金淋巴瘤。
