Department of Hematology, Amsterdam UMC, University of Amsterdam, Cancer Center Amsterdam and LYMMCARE (Lymphoma and Myeloma Center Amsterdam), Amsterdam, The Netherlands.
Department of Pathology, Amsterdam UMC, University of Amsterdam, Cancer Center Amsterdam and LYMMCARE (Lymphoma and Myeloma Center Amsterdam), Amsterdam, The Netherlands.
Leuk Lymphoma. 2022 May;63(5):1070-1079. doi: 10.1080/10428194.2021.2018584. Epub 2021 Dec 27.
Discrimination between Waldenström macroglobulinemia (WM) and marginal zone lymphoma (MZL) of the bone marrow (BM) can be difficult due to overlap in clinical, histopathologic, and immunophenotypic characteristics. We determined which characteristics can aid in the differential diagnosis of 'gray zone' cases. We compared clinical, histopathologic, immunophenotypic, and molecular features of 222 WM and 65 MZL patients. LASSO regression was employed for variable selection. The most distinguishing clinical features of WM compared to MZL were the presence of the B-symptom weight loss and IgM paraprotein. Histopathological findings were plasmacytoid differentiation, monoclonal plasma cells, and increased mast cells in the BM. Regarding flow cytometry, only CD10 and CD38 were distinguishing markers. Finally, as the expected presence of the MYD88 mutation showed to be of great value in the distinction between WM and MZL. Despite the great overlap, WM can often be distinguished from MZL by using a combination of characteristics. These characteristics should be weighed in complex, 'gray zone' cases.
由于瓦尔登斯特伦巨球蛋白血症 (WM) 和骨髓 (BM) 边缘区淋巴瘤 (MZL) 之间在临床、组织病理学和免疫表型特征上存在重叠，因此鉴别这两者可能具有一定难度。我们确定了哪些特征可以帮助对“灰色区域”病例进行鉴别诊断。我们比较了 222 例 WM 和 65 例 MZL 患者的临床、组织病理学、免疫表型和分子特征。采用 LASSO 回归进行变量选择。与 MZL 相比，WM 的最显著临床特征为 B 症状体重减轻和 IgM 单克隆丙种球蛋白。组织病理学发现为骨髓中浆细胞样分化、单克隆浆细胞和肥大细胞增多。关于流式细胞术，只有 CD10 和 CD38 是具有鉴别意义的标志物。最后，MYD88 突变的预期存在表明在 WM 和 MZL 的鉴别中具有重要价值。尽管存在很大的重叠，但 WM 通常可以通过组合使用特征与 MZL 区分开来。这些特征应在复杂的“灰色区域”病例中进行权衡。
