伯基特淋巴瘤的免疫表型变异。

Immunophenotypic variations of Burkitt lymphoma.

机构信息

Dept of Pathology, Oregon Health & Science University, Portland, 97239-3098, USA.

出版信息

Am J Clin Pathol. 2010 Jul;134(1):127-38. doi: 10.1309/AJCP93LJPTRQPDKR.

PMID:20551277

Abstract

Burkitt lymphoma (BL) exhibits a characteristic immunophenotype that is positive for pan-B-cell antigens and CD10 and expresses clonal surface immunoglobulins (SIgs). We evaluated 35 BLs and identified atypical immunophenotypes in 4 including SIg light chain negativity in 4, negativity for B-cell antigens in 2, lack of CD10 in 1, and CD4 expression in 1. All 4 cases showed morphologic features characteristic of BL, and all were confirmed by cytogenetic analysis. The 4 BL cases included 1 girl, 2 men with HIV/AIDS, and a third man with a history of heart transplantation. Two patients died shortly after diagnosis; the other 2 completed hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone with highly active antiretroviral therapy and achieved complete remission. Our study indicates that an unusual immunophenotype is not uncommon in BL. If the immunophenotype is interpreted in isolation, the diagnosis could be missed. Recognizing the variability of immunophenotype is essential for establishing an accurate diagnosis of BL.

摘要

伯基特淋巴瘤（BL）表现出特征性的免疫表型，对泛 B 细胞抗原和 CD10 呈阳性，表达克隆性表面免疫球蛋白（SIgs）。我们评估了 35 例 BL，并在其中 4 例中发现了非典型免疫表型，包括 4 例 SIg 轻链阴性、2 例 B 细胞抗原阴性、1 例 CD10 缺失和 1 例 CD4 表达。所有 4 例病例均表现出典型的 BL 形态学特征，并均通过细胞遗传学分析得到证实。这 4 例 BL 病例包括 1 例女孩、2 例 HIV/AIDS 男性患者和 1 例有心脏移植史的男性患者。2 例患者在诊断后不久死亡；另外 2 例患者在接受高度活跃的抗逆转录病毒治疗和超分割环磷酰胺、长春新碱、多柔比星和地塞米松治疗后达到完全缓解。我们的研究表明，BL 中不常见不典型免疫表型。如果孤立地解释免疫表型，可能会漏诊。认识到免疫表型的可变性对于建立 BL 的准确诊断至关重要。