Department of Cellular and Molecular Biology and Pathology, Federico II University of Naples, Italy.
Int J Immunopathol Pharmacol. 2010 Jan-Mar;23(1):307-16. doi: 10.1177/039463201002300129.
Thymomas are rare tumours that sustain T-lymphopoiesis and trigger a variety of autoimmune diseases and immunodeficiencies, including a fatal hypogammaglobulinemia, namely Goods Syndrome (GS). Due to its rarity, GS has been poorly investigated and immunological features, as well as pathogenetic mechanisms underlying this syndrome, are unclear. We studied 30 thymoma patients by performing an immunological assessment, including immunophenotype and analysis of T cell repertoire (TCR). Development of GS was characterized by a progressive decrease in B, CD4 T and NK lymphocytes. These alterations paired with accumulation of CD8+CD45RA+ T cells that showed a polyclonal repertoire without expansions of specific clonotypes. GS is defined as hypogammaglobulinemia with thymoma. Here, we show for the first time that this syndrome is characterized by a severe loss of CD4+, NK and B cells. Furthermore, the accumulation of CD8+CD45RA+ T lymphocytes parallels these changes; this accumulation may have a role in determining the disease and can be used to monitor clinical stages of immunodeficiency in thymoma.
胸腺瘤是一种罕见的肿瘤，它能维持 T 淋巴细胞生成，并引发多种自身免疫性疾病和免疫缺陷，包括致命性低丙种球蛋白血症，即 Good 综合征（GS）。由于其罕见性，GS 研究不足，其免疫学特征和发病机制尚不清楚。我们通过进行免疫学评估，包括免疫表型和 T 细胞受体（TCR）分析，研究了 30 例胸腺瘤患者。GS 的发展特征是 B、CD4 T 和 NK 淋巴细胞逐渐减少。这些改变与 CD8+CD45RA+T 细胞的积累相吻合，这些细胞表现出多克隆 repertoire，没有特定克隆型的扩增。GS 定义为伴有胸腺瘤的低丙种球蛋白血症。在这里，我们首次表明，这种综合征的特征是 CD4+、NK 和 B 细胞的严重缺失。此外，CD8+CD45RA+T 淋巴细胞的积累与这些变化平行；这种积累可能在决定疾病方面发挥作用，并可用于监测胸腺瘤免疫缺陷的临床阶段。
