T/NK 大颗粒淋巴细胞白血病伴发类似单克隆 B 细胞淋巴增生性疾病：一种未被认识但却频繁相关的疾病。

T/NK large granular lymphocyte leukemia and coexisting monoclonal B-cell lymphocytosis-like proliferations. An unrecognized and frequent association.

机构信息

Department of Clinical Pathology and Taussig Cancer Center, Cleveland Clinic, Cleveland, OH, USA.

出版信息

Am J Clin Pathol. 2010 Jun;133(6):936-41. doi: 10.1309/AJCPIIL1Y5QXTIBP.

PMID:20472852

Abstract

T-cell large granular lymphocyte leukemia (T-LGLL) is a T-cell lymphoproliferative disorder that has recently been associated with B-cell dyscrasias on a spectrum ranging from dysgammaglobulinemia to lymphoma. To investigate the relationship between clonal B-cell and LGLL lymphoproliferations, we systematically studied lymphocytes in 57 patients with T-LGLL or NK lymphocytosis using flow cytometric methods sensitive to low-level B-cell populations. We identified 16 patients (28%) with abnormal B-cell populations; 9 (16%) of the patients had no known history of a B-cell lymphoproliferative disorder. We characterized these abnormal B-cell populations as monoclonal B-cell lymphocytosis and report a high frequency of monoclonal B-cell lymphocytosis in T/NK LGLL. Our findings suggest that certain pathologic factors may operate in patients with T/NK LGLL to drive low-level clonal B-cell proliferations.

摘要

T 细胞大颗粒淋巴细胞白血病（T-LGLL）是一种 T 细胞淋巴增生性疾病，最近与从低丙种球蛋白血症到淋巴瘤的一系列 B 细胞异常相关联。为了研究克隆性 B 细胞与 LGLL 淋巴细胞增生之间的关系，我们使用流式细胞术方法对 57 例 T-LGLL 或 NK 淋巴细胞增多症患者的淋巴细胞进行了系统研究，这些方法对低水平 B 细胞群敏感。我们确定了 16 例（28%）患者存在异常 B 细胞群；其中 9 例（16%）患者无已知的 B 细胞淋巴增生性疾病史。我们将这些异常 B 细胞群描述为单克隆 B 细胞淋巴细胞增多症，并报告 T/NK LGLL 中单克隆 B 细胞淋巴细胞增多症的高频率。我们的研究结果表明，某些病理因素可能在 T/NK LGLL 患者中起作用，导致低水平克隆性 B 细胞增殖。