Lin C W, O'Brien S, Faber J, Manshouri T, Romaguera J, Huh Y O, Kantarjian H, Keating M, Albitar M
Department of Laboratory Medicine, University of Texas M. D. Anderson Cancer Center, Houston 77030, USA.
Am J Clin Pathol. 1999 Dec;112(6):828-35. doi: 10.1093/ajcp/112.6.828.
CD5 is a T-cell marker aberrantly expressed in B-cell chronic lymphocytic leukemia and mantle cell lymphoma. Other B-cell neoplasms, including Burkitt lymphoma, are usually CD5-. We report 4 cases of de novo CD5+ Burkitt lymphoma/leukemia in elderly patients, all of whom were in a leukemic phase and had variable lymph node and splenic involvement. The blasts were typically medium sized, with folded nuclei, distinct but not prominent nucleoli, and moderate amounts of somewhat vacuolated basophilic cytoplasm; they were terminal deoxynucleotidyl transferase--negative and surface immunoglobulin--positive. All 4 cases demonstrated c-myc rearrangement, but none had t(14;18), t(11;14), or cyclin D1 overexpression or rearrangement. Only 1 patient achieved complete remission after hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) therapy. One patient responded poorly to hyper-CVAD, and 2 patients died during induction chemotherapy. These rare cases of aggressive lymphoid malignancy with CD5 positivity and molecular features associated with Burkitt lymphoma/leukemia are best classified as Burkitt leukemia. However, the morphologic and immunophenotypic similarity to the blastoid variant of mantle cell lymphoma are diagnostically challenging. The diseases can be distinguished at the genetic level, since Burkitt lymphoma involves the rearrangement of c-myc, and mantle cell lymphoma usually the overexpression or rearrangement of cyclin D1.
CD5是一种T细胞标志物,在B细胞慢性淋巴细胞白血病和套细胞淋巴瘤中异常表达。其他B细胞肿瘤,包括伯基特淋巴瘤,通常不表达CD5。我们报告了4例老年患者的初发性CD5阳性伯基特淋巴瘤/白血病,所有患者均处于白血病期,伴有不同程度的淋巴结和脾脏受累。原始细胞通常中等大小,核呈折叠状,核仁明显但不突出,嗜碱性细胞质中等量且有一些空泡;它们末端脱氧核苷酸转移酶阴性,表面免疫球蛋白阳性。所有4例均显示c-myc重排,但均无t(14;18)、t(11;14)或细胞周期蛋白D1过表达或重排。只有1例患者在接受Hyper-CVAD(超分割环磷酰胺、长春新碱、阿霉素、地塞米松)治疗后达到完全缓解。1例患者对Hyper-CVAD反应不佳,2例患者在诱导化疗期间死亡。这些罕见的侵袭性淋巴恶性肿瘤病例具有CD5阳性以及与伯基特淋巴瘤/白血病相关的分子特征,最好归类为伯基特白血病。然而,其形态学和免疫表型与套细胞淋巴瘤母细胞样变异型相似,在诊断上具有挑战性。这两种疾病在基因水平上可以区分,因为伯基特淋巴瘤涉及c-myc重排,而套细胞淋巴瘤通常涉及细胞周期蛋白D1过表达或重排。