Qiu Lianqun, Wang Sa A, Tang Guilin, Wang Wei, Lin Pei, Xu Jie, Yin C Cameron, Khanlari Mahsa, Medeiros L Jeffrey, Li Shaoying
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Department of Laboratory Medicine & Pathology, University of Washington, Seattle, WA 98115, USA.
Cancers (Basel). 2023 Jan 30;15(3):848. doi: 10.3390/cancers15030848.
Blastoid B-cell neoplasms mainly include B-lymphoblastic leukemia/lymphoma (B-ALL), blastoid mantle cell lymphoma, and high-grade B-cell lymphoma with blastoid morphologic features (blastoid HGBL). Distinguishing blastoid HGBL from B-ALL can be challenging and we previously developed six-point flow cytometry-focused and three-point immunohistochemistry-focused scoring systems to aid in differential diagnosis. However, the six-point scoring system was derived from bone marrow cases and occasional cases may have a misleading score using either system. In this study, we assessed 121 cases of blastoid-HGBL (37 BM and 84 extramedullary) to validate the six-point scoring system in all tissue types and to further compare the two scoring systems. Compared with 47 B-ALL cases enriched for CD34-negative neoplasm, the 121 blastoid-HGBL cases showed distinctive pathologic features. The six-point scoring system showed a sensitivity of 100%. A comparison of the two scoring systems in blastoid HGBL ( = 64) and B-ALL ( = 37) showed a concordance score rate of 88%. Thirteen cases showed misleading scores, including five HGBL and eight B-ALL, and the diagnosis was further validated by gene transcriptome profiling. Twelve of thirteen cases had discordant scores between the two scoring systems. Simultaneous employment of both scoring systems improved the accuracy of classification of blastoid B-cell neoplasms to 99%. In conclusion, the previously defined six-point scoring system showed an excellent performance regardless of the tissue origin. Using both scoring systems together improves the accuracy of classification of blastoid B-cell neoplasms. Cases with discordant scores between the two scoring systems were extremely challenging neoplasms and classification required correlation with all available clinical and genetic features.
母细胞样B细胞肿瘤主要包括B淋巴细胞白血病/淋巴瘤（B-ALL）、母细胞样套细胞淋巴瘤以及具有母细胞样形态特征的高级别B细胞淋巴瘤（母细胞样HGBL）。将母细胞样HGBL与B-ALL区分开来可能具有挑战性，我们之前开发了一个六点流式细胞术为主的评分系统和一个三点免疫组化为主的评分系统来辅助鉴别诊断。然而，六点评分系统源自骨髓病例，偶尔会有病例使用任一系统时得分具有误导性。在本研究中，我们评估了121例母细胞样HGBL（37例骨髓和84例髓外），以在所有组织类型中验证六点评分系统，并进一步比较这两个评分系统。与47例富集CD34阴性肿瘤的B-ALL病例相比，121例母细胞样HGBL病例表现出独特的病理特征。六点评分系统显示敏感性为100%。在64例母细胞样HGBL和37例B-ALL中比较这两个评分系统，一致性评分率为88%。13例病例得分具有误导性，包括5例HGBL和8例B-ALL，通过基因转录组分析进一步验证了诊断。13例病例中有12例在两个评分系统之间得分不一致。同时使用这两个评分系统将母细胞样B细胞肿瘤的分类准确性提高到99%。总之，先前定义的六点评分系统无论组织来源如何均表现出色。同时使用这两个评分系统可提高母细胞样B细胞肿瘤的分类准确性。两个评分系统得分不一致的病例是极具挑战性的肿瘤，分类需要与所有可用的临床和基因特征相关联。
