Marlies Arnold, Udo Gaipl, Juergen Brunner, Bernd Spriewald, Herrmann Martin, Haas Johannes Peter
Department of Internal Medicine 3, Institute of Clinical Immunology and Rheumatology, University Hospital Erlangen, Erlangen, Germany.
Autoimmunity. 2007 Jun;40(4):299-301. doi: 10.1080/08916930701356473.
The autoimmune lymphoproliferative syndrome (ALPS) is characterized by non-malignant lymphoproliferation and signs of autoimmunity. A hallmark of ALPS are high amounts of circulating CD3+/CD4-/CD8- double negative T-lymphocytes (DN T cells). The origin of these cells remains elusive. To investigate the relationships of DN T cells and the single positive T cell populations (CD4+ and CD8+), we analyzed by spectratyping the complementarity determining regions 3 (CDR3) of the T cell receptors in sorted "single positive" (CD4+, CD8+) and DN T cells in a patient with ALPS type 1a. We observed signs for clonal expansion in all three T cell subpopulations. Strong and weak clonal expansions were to be seen in 16 and 14 for DN, 6 and 12 for CD8+, and 1 and 5 for CD4 + T cells, respectively. Most importantly, 24 out of 30 aberrant peaks in the spectratype histograms of the DN T cells where unique for this population and were not to be detected in the histograms of the single positive T cells. In contrast to published data, we conclude that expanded DN T cell populations in ALPS are not generally derived from expanded CD3+/CD4+ or CD3+/CD8+ populations.
自身免疫性淋巴细胞增生综合征（ALPS）的特征是良性淋巴细胞增生和自身免疫迹象。ALPS的一个标志是循环中存在大量CD3⁺/CD4⁻/CD8⁻双阴性T淋巴细胞（DN T细胞）。这些细胞的起源仍然不明。为了研究DN T细胞与单阳性T细胞群体（CD4⁺和CD8⁺）之间的关系，我们通过光谱分型分析了一名1a型ALPS患者中经分选的“单阳性”（CD4⁺、CD8⁺）和DN T细胞的T细胞受体互补决定区3（CDR3）。我们在所有三个T细胞亚群中都观察到了克隆扩增的迹象。DN T细胞中分别有16个和14个出现强克隆扩增和弱克隆扩增，CD8⁺ T细胞中分别有6个和12个，CD4⁺ T细胞中分别有1个和5个。最重要的是，DN T细胞光谱型直方图中的30个异常峰中有24个是该群体特有的，在单阳性T细胞的直方图中未检测到。与已发表的数据相反，我们得出结论，ALPS中扩增的DN T细胞群体通常并非源自扩增的CD3⁺/CD4⁺或CD3⁺/CD8⁺群体。
