Department of Pathology, Basic Medical College, Capital Medical University, Beijing, China.
Leuk Lymphoma. 2011 Dec;52(12):2356-64. doi: 10.3109/10428194.2011.602772. Epub 2011 Aug 18.
Differential diagnosis of Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL) and B cell lymphoma, unclassifiable, with features intermediate between BL and DLBCL (intermediate BL/DLBCL) can be difficult. We studied 97 Chinese pediatric mature aggressive B-cell lymphomas including 81 BL cases, eight DLBCL cases and eight intermediate BL/DLBCL cases using immunohistochemistry, interphase fluorescence in situ hybridization (FISH) and Epstein-Barr virus (EBV) in situ hybridization. Our results showed that there were no significant differences in the expression of CD10 and BCL6 among cases of BL (91% and 86%, respectively), DLBCL (75% and 63%, respectively) and intermediate BL/DLBCL (75% and 63%, respectively) (p > 0.05). The expression of BCL2 (3% in BL, 50% in DLBCL, 50% in intermediate BL/DLBCL), expression of MUM1 (17% in BL, 63% in DLBCL, 63% in intermediate BL/DLBCL) and mean Ki-67 proliferation index (PI) (93% in BL, 83% in DLBCL, 80% in intermediate BL/DLBCL) were significantly different between BL and DLBCL and between BL and intermediate BL/DLBCL. The frequency of an extra copy of BCL6 (0% in BL, 37.5% in DLBCL, 25% in intermediate BL/DLBCL) and EBV-encoded RNA (EBER) positivity (48% in BL, 0% in DLBCL and intermediate BL/DLBCL) were also significantly different between BL and DLBCL and between BL and intermediate BL/DLBCL. The frequency of C-MYC rearrangement in BL (98%) was much higher than in DLBCL (37.5%) and intermediate BL/DLBCL (50%). Our findings suggest that diagnosis of the mature aggressive B-cell lymphomas in pediatrics should be based on the comprehensive review and integration of morphological, immunohistochemical and molecular genetic features. The expression of CD10 and BCL6 but not BCL2, a high Ki-67 PI (>90%) and a C-MYC rearrangement but not BCL2 or BCL6 rearrangement are the features of BL. MUM1 is not an exclusionary diagnostic marker for BL. As the immunophenotype and molecular genetic features of DLBCL and intermediate BL/DLBCL are similar, intermediate BL/DLBCL is more likely a subgroup of DLBCL in the pediatric population. Regardless of the expression of CD10 and BCL6, strong staining for BCL2, Ki-67 PI below 90% and the presence of extra copies of BCL6 favor a diagnosis of DLBCL or intermediate BL/DLBCL.
伯基特淋巴瘤 (BL)、弥漫性大 B 细胞淋巴瘤 (DLBCL) 和 B 细胞淋巴瘤，分类介于两者之间，具有 BL 和 DLBCL 之间的特征（中间 BL/DLBCL）的鉴别诊断可能具有一定难度。我们使用免疫组织化学、间期荧光原位杂交 (FISH) 和 EBV 原位杂交研究了 97 例中国儿科成熟侵袭性 B 细胞淋巴瘤，包括 81 例 BL 病例、8 例 DLBCL 病例和 8 例中间 BL/DLBCL 病例。我们的研究结果表明，BL（分别为 91%和 86%）、DLBCL（分别为 75%和 63%）和中间 BL/DLBCL（分别为 75%和 63%）病例中 CD10 和 BCL6 的表达没有显著差异（p > 0.05）。BCL2 的表达（BL 中为 3%，DLBCL 中为 50%，中间 BL/DLBCL 中为 50%）、MUM1 的表达（BL 中为 17%，DLBCL 中为 63%，中间 BL/DLBCL 中为 63%）和平均 Ki-67 增殖指数（PI）（BL 中为 93%，DLBCL 中为 83%，中间 BL/DLBCL 中为 80%）在 BL 和 DLBCL 之间以及 BL 和中间 BL/DLBCL 之间存在显著差异。BCL6 额外拷贝的频率（BL 中为 0%，DLBCL 中为 37.5%，中间 BL/DLBCL 中为 25%）和 EBV 编码 RNA（EBER）阳性率（BL 中为 48%，DLBCL 和中间 BL/DLBCL 中为 0%）在 BL 和 DLBCL 之间以及 BL 和中间 BL/DLBCL 之间也存在显著差异。BL 中 C-MYC 重排的频率（98%）远高于 DLBCL（37.5%）和中间 BL/DLBCL（50%）。我们的研究结果表明，儿科侵袭性 B 细胞淋巴瘤的诊断应基于形态学、免疫组织化学和分子遗传学特征的综合评估。CD10 和 BCL6 的表达，而不是 BCL2 的表达、高 Ki-67 PI（>90%）和 C-MYC 重排，但不是 BCL2 或 BCL6 重排，是 BL 的特征。MUM1 不是 BL 的排除性诊断标志物。由于 DLBCL 和中间 BL/DLBCL 的免疫表型和分子遗传学特征相似，中间 BL/DLBCL 更可能是儿科人群中 DLBCL 的一个亚组。无论 CD10 和 BCL6 的表达如何，BCL2 强染色、Ki-67 PI 低于 90%和 BCL6 额外拷贝的存在均提示诊断为 DLBCL 或中间 BL/DLBCL。
