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小B细胞性淋巴瘤各个亚型具有相似细胞形态，而生物学行为、预后和治疗方式却往往迥异，其鉴别诊断有重要的临床意义。典型的CLL/SLL和MCL除了CD5＋CD10－为其共同免疫表型外，对于某些抗原，两者的表达相反，例如CD20、CD22、CD79b、CD11c、sIg、CD23、FMC7等。然而并非所有CLL/SLL和MCL都具有如此典型的表型。
对于CLL/SLL已有相关的诊断评分系统，基于CD5、CD79b、CD23、FMC7、sIg（Moreau and Matutes et al., 1997）。
对于套细胞淋巴瘤，Medd等人建立了评分系统，血小板计数<150×10E9/L，CD23－、CD20强＋、CD38＋各计1分，MCL均>=3分。
A Novel Scoring System Combining Expression of CD23, CD20, and CD38 with Platelet Count Predicts for the Presence of the t(11;14) Translocation of Mantle Cell Lymphoma Background: Both chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL) are CD5/19 positive. The t(11;14) MCL translocation is identified by fluorescent in situ hybridization (FISH) and can distinguish the two disorders. We attempted to identify flow cytometric and other markers predictive of a positive FISH test.
Methods: We examined 100 atypical CLL/MCL cases for demographic, hematological, and cytometric variables, 96 were FISH tested for t(11;14) and four were known MCL.
Results: Twenty-two cases were confirmed as MCL. Multivariate analysis identified four variables associated with MCL: Thrombocytopenia (taken as Plt < 150 3 109/L), CD23 negative, CD20 strong, and CD38 positive, with these variables a four-point score was devised. By ROC analysis, the MCL score was superior in differentiating MCL to the Marsden CLL score (AUC 0.95 vs. 0.78). MCL score 2 showed sensitivity 1, specificity 0.66, positive predictive value (PPV) 0.49, and negative predictive value (NPV) 1 for MCL. The score was then prospectively validated on an independent cohort of 44 cases of atypical CLL/MCL. No MCL had a score <3. Validation PPV/NPV of score 3 were 0.5/1. Overall survival in MCL was shorter compared to t(11;14) negative patients (median 3.3 vs. 4.2 years, HR 2.2, 95% CI 0.87–5.5, P 5 0.1).
Conclusions: The score described can be used to identify cases of CD5/19 positive lymphoproliferative disorders likely to be t(11;14) positive MCL.
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发表于 2012-7-27 14:02:28
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