Gorodetskiy Vadim R, Probatova Natalya A, Kupryshina Natalia A, Palshina Svetlana G, Obukhova Tatiana N, Sidorova Yulia V, Ryzhikova Natalya V, Sudarikov Andrey B
Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Moscow, Russia.
Cancer Manag Res. 2020 Sep 30;12:9449-9457. doi: 10.2147/CMAR.S261910. eCollection 2020.
The peculiar features of T-cell large granular lymphocytic leukemia (T-LGLL) are its association with autoimmune disorders (particularly with rheumatoid arthritis (RA)) and a broad spectrum of B-cell lymphoproliferative disorders. However, association of T-LGLL with mantle cell lymphoma (MCL) is extremely rare. Here, we describe a case of an 80-year-old man admitted with suspected Felty's syndrome. The blood count showed white blood cells at 2.2×10/L, with 3% neutrophils, 88% lymphocytes, and at 0.66×10/L LGLs. The spleen had been removed 43 months prior to the admission due to suspected B-cell splenic lymphoma. Re-examination of the spleen revealed cyclin D1+ and SOX11- lymphocytes in the inner part of the unexpanded mantle zones of the white pulp follicles, thus displaying a so-called in situ histologic pattern of MCL, and in small clusters in the red pulp. The splenic cords were moderately expanded by lymphocytes expressing CD3, TIA1, and granzyme B but not CD4 and CD8. Monoclonal rearrangements of the gene and the genes, polyclonal rearrangements of the gene, mutation of the gene (c.1940A>T; p.N647I), and t(11;14)(q13;q32) translocation were identified in the spleen sample. Flow cytometry of bone marrow revealed a population of TCR γδ+, CD3+, CD4-, CD5-, CD7+, CD8-, CD16-, CD56-, and CD57- lymphocytes. Fragment analysis demonstrated identical gene clonal rearrangement patterns in the spleen and bone marrow samples. In this study, we describe the first case of simultaneous presentation of γδ T-LGLL and leukemic non-nodal MCL (L-NN-MCL) in a patient with RA and present morphological findings of L-NN-MCL in the spleen.
T 细胞大颗粒淋巴细胞白血病（T-LGLL）的独特特征在于其与自身免疫性疾病（尤其是类风湿关节炎（RA））以及广泛的 B 细胞淋巴增殖性疾病相关。然而，T-LGLL 与套细胞淋巴瘤（MCL）的关联极为罕见。在此，我们描述一例 80 岁男性因疑似费尔蒂综合征入院的病例。血常规显示白细胞计数为 2.2×10⁹/L，中性粒细胞占 3%，淋巴细胞占 88%，大颗粒淋巴细胞（LGL）占 0.66×10⁹/L。入院前 43 个月因疑似 B 细胞脾脏淋巴瘤已切除脾脏。脾脏重新检查显示，在白髓滤泡未扩张的套区内部存在细胞周期蛋白 D1 阳性且 SOX11 阴性的淋巴细胞，呈现出所谓的 MCL 原位组织学模式，在红髓中则呈小簇状分布。脾索被表达 CD3、TIA1 和颗粒酶 B 但不表达 CD4 和 CD8 的淋巴细胞中度扩张。在脾脏样本中鉴定出 基因和 基因的单克隆重排、 基因的多克隆重排、 基因（c.1940A>T；p.N647I）突变以及 t(11;14)(q13;q32)易位。骨髓流式细胞术显示一群 TCRγδ⁺、CD3⁺、CD4⁻、CD5⁻、CD7⁺、CD8⁻、CD16⁻、CD56⁻和 CD57⁻淋巴细胞。片段分析显示脾脏和骨髓样本中 基因克隆重排模式相同。在本研究中，我们描述了首例 RA 患者同时出现γδ T-LGLL 和白血病非淋巴结 MCL（L-NN-MCL）的病例，并展示了脾脏中 L-NN-MCL 的形态学表现。
