免疫性血小板减少症患者中巨大颗粒淋巴细胞的发生率及意义。
Prevalence and significance of large granular lymphocytes in patients with immune thrombocytopenia.
机构信息
McMaster Centre for Transfusion Research, Department of Medicine, McMaster University, Hamilton, ON, Canada and.
Hamilton Health Sciences, Juravinski site, Hamilton, ON, Canada.
出版信息
Platelets. 2023 Dec;34(1):2144194. doi: 10.1080/09537104.2022.2144194.
The association between T-cell large granular lymphocytes (T-LGL) and ITP is uncertain. The aims of this study were to determine the prevalence of T-LGL in patients with ITP and to describe its association with ITP disease severity. We analyzed flow cytometry results for T-LGL (using a threshold of 0.3 x10 or greater cells/L) or positive T-cell receptor clonality in patients with ITP and nonimmune thrombocytopenia. Descriptive statistics were used to characterize the association between T-LGL and ITP, response to ITP treatments (rituximab and splenectomy) and response to T-LGL treatment. Among ITP patients, 14.3% (13/91) had evidence of a T-LGL population compared to 10.3% (3/29) of patients with non-immune thrombocytopenia. ITP patients with T-LGL had lower nadir platelet counts (2 vs. 47 × 10/L) and received more ITP treatments (median 6 vs. 3) than ITP patients without T-LGL. Response to rituximab was observed in 14.3% (1/7) of ITP patients with T-LGL and 54.5% (6/11) without T-LGL. Response to splenectomy was observed in 25% (2/8) with T-LGL and 56.2% (9/16) without T-LGL. Four patients with ITP and T-LGL received treatment for T-LGL with methotrexate; none had an improvement in platelet count levels. T-LGL may appear in patients with ITP, and the meaning of this finding remains unclear; however, for some patients, the presence of abnormal T-LGL may indicate a more severe form of ITP that tends to be less responsive to therapy. In this cohort, treatment of T-LGL with methotrexate did not improve platelet counts in the few patients who were treated.
T 细胞大颗粒淋巴细胞（T-LGL）与 ITP 之间的关联尚不确定。本研究旨在确定 T-LGL 在 ITP 患者中的患病率，并描述其与 ITP 疾病严重程度的关系。我们分析了 ITP 患者和非免疫性血小板减少症患者的 T-LGL（使用 0.3x10 或更高细胞/L 的阈值）或阳性 T 细胞受体克隆性的流式细胞术结果。描述性统计用于描述 T-LGL 与 ITP、ITP 治疗（利妥昔单抗和脾切除术）反应和 T-LGL 治疗反应之间的关系。在 ITP 患者中，有 14.3%（13/91）存在 T-LGL 群体，而在非免疫性血小板减少症患者中，有 10.3%（3/29）存在 T-LGL 群体。与无 T-LGL 的 ITP 患者相比，有 T-LGL 的 ITP 患者的血小板计数最低值更低（2 对 47×10/L），接受的 ITP 治疗更多（中位数 6 对 3）。有 T-LGL 的 ITP 患者中，有 14.3%（1/7）对利妥昔单抗有反应，而无 T-LGL 的 ITP 患者中有 54.5%（6/11）对利妥昔单抗有反应。有 T-LGL 的 ITP 患者中有 25%（2/8）对脾切除术有反应，而无 T-LGL 的 ITP 患者中有 56.2%（9/16）对脾切除术有反应。4 例 ITP 和 T-LGL 患者接受甲氨蝶呤治疗 T-LGL；无一例血小板计数水平改善。T-LGL 可能出现在 ITP 患者中，其发现的意义尚不清楚；然而，对于一些患者来说，异常 T-LGL 的存在可能表明 ITP 更为严重，且对治疗的反应较差。在本队列中，用甲氨蝶呤治疗 T-LGL 并没有改善少数接受治疗的患者的血小板计数。
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