Fang Hong, Medeiros L Jeffrey, Tang Zhenya, Wang Wei, Ok Chi Y, Patel Keyur P, Khoury Joseph D, Thakral Beenu
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States of America.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States of America.
Ann Diagn Pathol. 2021 Feb;50:151649. doi: 10.1016/j.anndiagpath.2020.151649. Epub 2020 Oct 24.
Leukemic, non-nodal mantle cell lymphoma (MCL) is a distinct, rare, indolent variant of mantle cell lymphoma, but can relapse aggressively. It can present with lymphocytosis with chronic lymphocytic leukemia (CLL)-like morphologic and immunophenotypic features as was initially considered in the index case. However, at time of splenectomy, two years later cyclin D1 overexpression was shown and the disease was realized to be leukemic non-nodal MCL. The patient was followed for 21 years, without therapy, before he developed clinically aggressive MCL with lymphadenopathy. Lymph node biopsy showed MCL, pleomorphic variant. We review the literature and discuss the features of leukemic non-nodal MCL as well as the potential pitfalls in diagnosis. Furthermore, we are not aware of another cases reported with a 21 year interval from initial diagnosis of leukemic non-nodal MCL to aggressive MCL.
白血病性非淋巴结套细胞淋巴瘤（MCL）是套细胞淋巴瘤的一种独特、罕见且惰性的变异型，但可发生侵袭性复发。它可表现为淋巴细胞增多，具有慢性淋巴细胞白血病（CLL）样的形态学和免疫表型特征，正如索引病例最初所考虑的那样。然而，两年后脾切除时显示细胞周期蛋白D1过表达，该疾病被确诊为白血病性非淋巴结MCL。该患者在未接受治疗的情况下随访了21年，之后发展为伴有淋巴结病的临床侵袭性MCL。淋巴结活检显示为多形性变异型MCL。我们回顾了文献并讨论了白血病性非淋巴结MCL的特征以及诊断中的潜在陷阱。此外，我们未发现有从白血病性非淋巴结MCL初始诊断到侵袭性MCL间隔21年的其他病例报道。
