Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Cancer. 2012 Mar 15;118(6):1566-73. doi: 10.1002/cncr.26433. Epub 2011 Aug 31.
B-cell lymphoma, Unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, for convenience referred to here as unclassifiable B-cell lymphoma, is a category in the 2008 World Health Organization system used for a group of histologically aggressive neoplasms that are difficult to classify definitively. Currently, there is no established standard therapy for these neoplasms.
The authors assessed MYC status and correlated it with treatment response and outcome in a group of 52 patients with unclassifiable B-cell lymphoma treated with either a standard DLBCL regimen (R-CHOP [rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone-related therapy]) or more intensive regimens, such as R-hyper-CVAD (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine). The regimens were selected by the treating clinicians based on the overall clinical and pathological findings.
Thirty (58%) unclassifiable B-cell lymphomas had MYC abnormalities (MYC(+) ) including 27 with rearrangement, 2 with amplification, and 1 with both. The MYC(+) and MYC(-) groups were similar in their age distribution and International Prognostic Index scores. Progression-free survival of patients with MYC(+) unclassifiable B-cell lymphoma treated initially with R-CHOP was significantly worse than patients treated with R-hyper-CVAD (P = .0358). In contrast, for the MYC(-) unclassifiable B-cell lymphoma group, some patients responded to R-CHOP, and others were refractory to R-hyper-CVAD.
MYC aberrations are common in unclassifiable B-cell lymphoma. The presence of MYC aberrations identifies a patient subset that requires more aggressive therapy than R-CHOP. In contrast, MYC(-) unclassifiable B-cell lymphoma patients responded variably to either R-CHOP or aggressive therapy, and the latter showed no survival advantage.
B 细胞淋巴瘤，无分类伴有弥漫性大 B 细胞淋巴瘤（DLBCL）和伯基特淋巴瘤之间特征，为方便起见，这里称为无分类 B 细胞淋巴瘤，是 2008 年世界卫生组织系统中用于一组组织学侵袭性肿瘤的类别，这些肿瘤难以明确分类。目前，这些肿瘤没有既定的标准治疗方法。
作者评估了 MYC 状态，并在一组 52 例无分类 B 细胞淋巴瘤患者中进行了治疗反应和结果的相关性评估，这些患者接受了标准的 DLBCL 方案（R-CHOP [利妥昔单抗加环磷酰胺、多柔比星、长春新碱和泼尼松相关治疗]）或更强化的方案，如 R-hyper-CVAD（利妥昔单抗加超分割环磷酰胺、长春新碱、多柔比星和地塞米松与高剂量甲氨蝶呤和阿糖胞苷交替）。这些方案是由治疗医生根据整体临床和病理发现选择的。
30 例（58%）无分类 B 细胞淋巴瘤存在 MYC 异常（MYC(+)），包括 27 例存在易位、2 例存在扩增和 1 例存在两者。MYC(+)和 MYC(-)组在年龄分布和国际预后指数评分方面相似。最初接受 R-CHOP 治疗的 MYC(+)无分类 B 细胞淋巴瘤患者的无进展生存期明显差于接受 R-hyper-CVAD 治疗的患者（P =.0358）。相比之下，对于 MYC(-)无分类 B 细胞淋巴瘤组，一些患者对 R-CHOP 有反应，而另一些患者对 R-hyper-CVAD 有抗药性。
MYC 异常在无分类 B 细胞淋巴瘤中很常见。MYC 异常的存在确定了需要比 R-CHOP 更积极治疗的患者亚组。相比之下，MYC(-)无分类 B 细胞淋巴瘤患者对 R-CHOP 或强化治疗反应不一，后者没有生存优势。
