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3例原发性脾弥漫性大B细胞淋巴瘤临床分析

[Clinical analysis of 3 cases with primary splenic diffuse large B-cell lymphoma].

作者信息

Tanaka Masaru, Tsunoda Saburo, Inoue Koichi, Izumi Tooru, Yamamoto Takanobu, Hoshi Sayuri, Hirabayashi Kaoru, Igarashi Seiji, Akutsu Miyuki, Kano Yasuhiko

机构信息

Division of Hematology, Tochigi Cancer Center.

出版信息

Rinsho Ketsueki. 2011 Aug;52(8):703-7.

Abstract

The definition of primary splenic lymphoma is controversial, but it has been reported to be a rare disease that comprises less than 1% of all malignant lymphomas. Three cases of primary splenic diffuse large B-cell lymphoma treated at our institution are described here. Median follow-up was 34.6 months (range 8.7∼39.2) and median age at diagnosis was 72 years old (range 65∼73). In all three cases, the diagnosis was definitively established not by splenectomy but by ultrasonically guided percutaneous splenic tissue core biopsy. Using the Hans classifier, one of the cases was subclassified as the germinal center B-cell like (GCB) subtype and two as non-GCB subtype. One case was CD5-positive diffuse large B-cell lymphoma. Two patients were in Ann Arbor stage II and one was in stage III. Using the International Prognostic Index, one was categorized as Low/intermediate risk, one as high/intermediate risk, and one as high risk. All patients underwent eight cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisolone followed by irradiation therapy. These three patients attained complete response. Although the follow-up period to date has been short, all patients have maintained a complete response and are currently alive. To determine whether our management protocol is valid, further observations are needed.

摘要

原发性脾淋巴瘤的定义存在争议,但据报道它是一种罕见疾病,在所有恶性淋巴瘤中所占比例不到1%。本文描述了在我们机构接受治疗的3例原发性脾弥漫性大B细胞淋巴瘤病例。中位随访时间为34.6个月(范围8.7至39.2个月),诊断时的中位年龄为72岁(范围65至73岁)。在所有3例病例中,确诊并非通过脾切除术,而是通过超声引导下经皮脾组织芯针活检。使用汉斯分类器,其中1例被亚分类为生发中心B细胞样(GCB)亚型,2例为非GCB亚型。1例为CD5阳性弥漫性大B细胞淋巴瘤。2例患者处于Ann Arbor分期II期,1例处于III期。根据国际预后指数,1例被归类为低/中危,1例为高/中危,1例为高危。所有患者均接受了8个周期的利妥昔单抗联合环磷酰胺、阿霉素、长春新碱和泼尼松龙治疗,随后进行放射治疗。这3例患者均达到完全缓解。尽管迄今为止的随访期较短,但所有患者均维持完全缓解,目前仍存活。为确定我们的治疗方案是否有效,还需要进一步观察。

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