de Leval Laurence, Braaten Kristina M, Ancukiewicz Marek, Kiggundu Edward, Delaney Thomas, Mankin Henry J, Harris Nancy Lee
Department of Pathology, Massachusetts General Hospital, Boston, 02114, USA.
Am J Surg Pathol. 2003 Sep;27(9):1269-77. doi: 10.1097/00000478-200309000-00011.
Twenty-nine patients with diffuse large B-cell lymphomas presenting with bone involvement, including 18 localized primary bone lymphomas (group 1), 2 multifocal primary bone lymphomas (group 2), and 9 patients with extraskeletal disease at diagnosis (group 3), were studied. The tumors were subclassified according to the criteria of the WHO classification and evaluated by immunohistochemistry for expression of antigens associated with germinal center (GC) and non-GC stages of B-cell differentiation (bcl-6, CD10, MUM-1, VS38c, CD138, bcl-2, and CD44). The presence of a BCL-2/IgH gene rearrangement was investigated by polymerase chain reaction. All cases were characterized by similar clinicopathologic and morphologic features and had similarly good overall outcome. The patients (23 males, 6 females, median age 44 years) had tumors in long bones (14), axial skeleton (8), limb girdles (3), and multiple sites (4). Most tumors (24) were centroblastic, with multilobated cells in 12 cases. Almost half of the tumors (14 of 29, 48%) were bcl-6+CD10+ (GC-like), 9 of 29 cases (31%) were bcl-6+CD10- (indeterminate phenotype), and 6 of 29 cases (21%) were CD10-bcl-6- (post-GC like). The indeterminate phenotype was seen only in primary bone lymphoma. MUM-1 was frequently expressed in GC-like and non-GC-like categories. We found no evidence of plasmacytic differentiation by CD138, and VS38c immunoreactivity was distinctly rare (2 of 29 cases). CD44 was detected in 6 tumors, all CD10-. Bcl-2 was expressed by 70% of the tumors, but only 1 of 23 cases tested had a Bcl-2/JH rearrangement by polymerase chain reaction. A survival analysis showed that GC-like tumors had a longer overall survival duration compared with non-GC-like tumors (P = 0.0046). In conclusion, a GC-like immunophenotype characterizes roughly half of large B-cell lymphomas of bone and is associated with an improved survival.
对29例出现骨受累的弥漫性大B细胞淋巴瘤患者进行了研究，其中包括18例局限性原发性骨淋巴瘤（第1组）、2例多灶性原发性骨淋巴瘤（第2组）以及9例诊断时存在骨外疾病的患者（第3组）。根据世界卫生组织分类标准对肿瘤进行亚分类，并通过免疫组织化学评估与B细胞分化生发中心（GC）和非GC阶段相关抗原（bcl-6、CD10、MUM-1、VS38c、CD138、bcl-2和CD44）的表达情况。采用聚合酶链反应研究BCL-2/IgH基因重排的存在情况。所有病例均具有相似的临床病理和形态学特征，总体预后同样良好。患者（23例男性，6例女性，中位年龄44岁）的肿瘤位于长骨（14例）、中轴骨骼（8例）、肢体带骨（3例）和多个部位（4例）。大多数肿瘤（24例）为中心母细胞性，12例具有多分叶细胞。几乎一半的肿瘤（29例中的14例，48%）为bcl-6+CD10+（GC样），29例中的9例（31%）为bcl-6+CD10-（不确定表型），29例中的6例（21%）为CD10-bcl-6-（GC后样）。不确定表型仅见于原发性骨淋巴瘤。MUM-1在GC样和非GC样类别中均频繁表达。我们未发现CD138提示浆细胞分化的证据，VS38c免疫反应性明显少见（29例中的2例）。在6例肿瘤中检测到CD44，均为CD10-。70%的肿瘤表达bcl-2，但通过聚合酶链反应检测，23例中仅1例存在Bcl-2/JH重排。生存分析显示，与非GC样肿瘤相比，GC样肿瘤的总生存时间更长（P = 0.0046）。总之，GC样免疫表型约占骨大B细胞淋巴瘤的一半，并与生存改善相关。
