Arinze Nkechi, Omar Nivin, Keruakous Amany, Kolhe Ravindra, Savage Natasha
Medical College of Georgia, Augusta University, Augusta, GA 30912, USA.
Hematol Rep. 2023 Oct 13;15(4):592-596. doi: 10.3390/hematolrep15040061.
Mantle cell lymphoma (MCL) is an intermediate-grade B-cell lymphoma, representing 2.8% of all non-Hodgkin lymphomas in the US. It is associated with t(11;14)(q13; q23), which leads to the overexpression of cyclin D1, consequently promoting cell proliferation. MCL usually expresses CD19, CD20, CD43, surface immunoglobulins, FMC7, BCL2, cyclin D1, CD5, and SOX11. Herein is a case of a 67-year-old male, referred to our facility with shortness of breath, anemia (hemoglobin of 5.3 g/dL), thrombocytopenia (12 × 10/L), and leukocytosis (283 × 10/L). A peripheral blood smear showed marked lymphocytosis with blastoid morphology. Morphologic examination of the bone marrow biopsy revealed a diffuse sheet of blastoid cells expressing CD20 and CD10, but without CD5 or cyclin D1. Given these features, a differential diagnosis of diffuse large B-cell lymphoma (DLBCL) with germinal center derivation, high-grade follicular lymphoma, and Burkitt lymphoma was considered, with the latter not favored due to morphology. Additional studies revealed positive SOX11, and fluorescence in situ hybridization (FISH) studies detected t(11;14). These additional studies supported diagnosis of the blastoid variant of MCL. In conclusion, we present a unique and challenging case of MCL without cyclin D1 or CD5, but with an expression of CD10 and SOX11, along with t(11;14). Pathologists should explicitly consider the blastoid variant of MCL when dealing with mature B-cell neoplasms with blastoid morphology in adults, and utilize a broad panel of ancillary studies, including FISH and SOX11.
套细胞淋巴瘤（MCL）是一种中等恶性程度的B细胞淋巴瘤，在美国所有非霍奇金淋巴瘤中占2.8%。它与t(11;14)(q13; q23)相关，该异常导致细胞周期蛋白D1过度表达，从而促进细胞增殖。MCL通常表达CD19、CD20、CD43、表面免疫球蛋白、FMC7、BCL2、细胞周期蛋白D1、CD5和SOX11。本文报告一例67岁男性，因呼吸急促、贫血（血红蛋白5.3 g/dL）、血小板减少（12×10⁹/L）和白细胞增多（283×10⁹/L）转诊至我院。外周血涂片显示有明显的母细胞样形态的淋巴细胞增多。骨髓活检的形态学检查显示一片弥漫的母细胞样细胞，表达CD20和CD10，但不表达CD5或细胞周期蛋白D1。基于这些特征，考虑鉴别诊断为具有生发中心来源的弥漫性大B细胞淋巴瘤（DLBCL）、高级别滤泡性淋巴瘤和伯基特淋巴瘤，由于形态学原因，后者可能性较小。进一步检查显示SOX11阳性，荧光原位杂交（FISH）研究检测到t(11;14)异常。这些进一步的检查支持了母细胞样变异型MCL的诊断。总之，我们报告了一例独特且具有挑战性的MCL病例，该病例不表达细胞周期蛋白D1或CD5，但表达CD10和SOX11，同时伴有t(11;14)异常。病理学家在处理成人具有母细胞样形态的成熟B细胞肿瘤时，应明确考虑母细胞样变异型MCL，并采用包括FISH和SOX11在内的一系列辅助检查。
