Richards M A, Hall P A, Gregory W M, Dhaliwal H S, Stansfeld A G, Amess J A, Lister T A
I.C.R.F. Department of Medical Oncology, St Bartholomew's Hospital, London, U.K.
Hematol Oncol. 1989 Jan-Feb;7(1):19-35. doi: 10.1002/hon.2900070103.
Lymphoplasmacytoid (lpc) and small cell centrocytic (scc) lymphoma are the two major sub-types of diffuse low grade non-Hodgkin's Lymphoma (NHL) within the Kiel classification. The presentation features and outcome for all 112 patients with these diagnoses (60 lpc, 52 scc) managed at St Bartholomew's Hospital between 1972 and 1986 are presented. The outcome for these patients is compared with that for patients with follicular and high grade lymphomas managed at this hospital during the same period. Nineteen of the 112 patients had localized (stages I-IIE) disease. In 18 of these cases the primary site of disease was extranodal, the gastrointestinal tract being involved in 12 cases. The survival for patients with localized disease was excellent. Eighteen are currently alive with median follow-up of 8 years. Ninety-three patients had advanced disease. A high incidence of splenomegaly, hepatomegaly, bone marrow and peripheral blood involvement was observed in both histological subgroups. A monoclonal paraprotein band was detected in the serum of nearly 50 per cent of patients with advanced lpc lymphoma. Patients with advanced disease were treated with either chlorambucil or cyclophosphamide, vincristine and prednisolone (CVP). The outcome was similar for both histological groups. Survival for these patients was poor (median 40 months) with less than 20 per cent surviving 5 years. Advanced age, elevated aspartate transaminase and failure to respond to treatment were identified by multivariate regression analysis as adverse prognostic factors.
淋巴浆细胞样(lpc)和小细胞中心细胞样(scc)淋巴瘤是基尔分类中弥漫性低度非霍奇金淋巴瘤(NHL)的两种主要亚型。本文介绍了1972年至1986年间在圣巴塞洛缪医院接受治疗的112例患有这些诊断的患者(60例lpc,52例scc)的临床表现和结局。将这些患者的结局与同期在该医院接受治疗的滤泡性和高度淋巴瘤患者的结局进行了比较。112例患者中有19例患有局限性(I-IIE期)疾病。在其中18例中,疾病的原发部位是结外,其中12例累及胃肠道。局限性疾病患者的生存率极佳。目前有18例存活,中位随访时间为8年。93例患者患有晚期疾病。在两个组织学亚组中均观察到脾肿大、肝肿大、骨髓和外周血受累的高发生率。在近50%的晚期lpc淋巴瘤患者血清中检测到单克隆副蛋白带。晚期疾病患者接受苯丁酸氮芥或环磷酰胺、长春新碱和泼尼松龙(CVP)治疗。两个组织学组的结局相似。这些患者的生存率较差(中位40个月),5年生存率不到20%。多因素回归分析确定高龄、天冬氨酸转氨酶升高和对治疗无反应是不良预后因素。