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伴有γδ T淋巴细胞亚群增多的古德综合征:一例报告。

Good's syndrome with increasing γδ T-lymphocyte subpopulation: A case report.

作者信息

Tadić Dragana, Marković Olivera, Kraguljac-Kurtović Nada, Drobnjak-Tomasek Olika

出版信息

Vojnosanit Pregl. 2015 Nov;72(11):1039-43. doi: 10.2298/vsp140609102t.

Abstract

INTRODUCTION

Good's syndrome is a rare cause of adult-onset immunodeficiency associated with thymoma. Good's syndrome should be considered in patients older than 40 years with the history of frequent infections. An abnormal immunoglobulin profile needs further investigation and flow cytometry which is crucial for establishing the diagno- sis of Good's syndrome.

CASE REPORT

We present a 56- year-old men with Good's syndrome diagnosed after a two-year history of recurrent infections. Examination of immune status of the patient showed decreased serum levels of all immunoglobulins. Flow cytometry of peripheral blood lym-phocyte revealed markedly reduced peripheral B cells, CD4 T-cell lymphopenia, inverted CD4/CD8 T-cell-ratio 0.37 (CD4--20.82%, CD8--70.7%). Analysis of the subpopulations of T-lymphocytes showed relative increasing γδ T cell receptor (TCR) T lymphocytes. Computed tomography scan of the chest showed a mediastinal mass compatible with thymoma of the diameter of 40 mm. After initiation of intravenous immunoglobulins the patient was in the good clinical condition and without bacterial complications. As the patient refused the operative treatment we continued to control the mediastinal tumor mass which did not increase during a 3-year follow-up.

CONCLUSION

The presented patient had a typical immunological finding for Good's syndrome, but also the increase in γδ TCR T-lymphocyte subpopulation for which it is difficult to determine whether this is pathogenetic or secondary reactive event.

摘要

引言

古德综合征是成人期免疫缺陷与胸腺瘤相关的罕见病因。40岁以上有频繁感染史的患者应考虑古德综合征。免疫球蛋白谱异常需要进一步检查,流式细胞术对于确诊古德综合征至关重要。

病例报告

我们报告一名56岁男性,在有两年反复感染病史后被诊断为古德综合征。对该患者免疫状态的检查显示所有免疫球蛋白的血清水平均降低。外周血淋巴细胞的流式细胞术显示外周B细胞明显减少,CD4 T细胞淋巴细胞减少,CD4/CD8 T细胞比值倒置为0.37(CD4为20.82%,CD8为70.7%)。T淋巴细胞亚群分析显示γδ T细胞受体(TCR)T淋巴细胞相对增加。胸部计算机断层扫描显示一个直径40mm的纵隔肿块,与胸腺瘤相符。静脉注射免疫球蛋白后,患者临床状况良好,无细菌并发症。由于患者拒绝手术治疗,我们继续监测纵隔肿瘤肿块,在3年随访期间其未增大。

结论

该患者有古德综合征典型的免疫学表现,但γδ TCR T淋巴细胞亚群也增加,难以确定这是致病事件还是继发反应性事件。

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