Van De Corput L, Falkenburg J H, Kluin-Nelemans J C
Department of Hematology, Laboratory of Experimental Hematology, Leiden University Medical Center, The Netherlands.
Leuk Lymphoma. 1998 Jun;30(1-2):31-9. doi: 10.3109/10428199809050927.
Hairy cell leukemia (HCL) is clinically associated with severe T-cell dysfunction. Several new observations have given more insight into the abnormal T-cell responses seen in this disease. T-lymphocytes in the spleen of patients with HCL seem to be abnormally activated. On the other hand, they are non-responsive, possibly as a result of monocytopenia which may lead to inadequate antigen presentation. This, together with the lack of CD28 on T-cells, may cause T-cell dysfunction. Furthermore, there is a very restricted repertoire of the T-cell receptor-beta family, which may also result in non-responsiveness. Otherwise, T-cell clonal excess may be indicative for activated, possibly autoreactive T-cells.
毛细胞白血病（HCL）在临床上与严重的T细胞功能障碍相关。一些新的观察结果使人们对这种疾病中异常的T细胞反应有了更深入的了解。HCL患者脾脏中的T淋巴细胞似乎被异常激活。另一方面，它们无反应，这可能是单核细胞减少导致抗原呈递不足的结果。这与T细胞上缺乏CD28一起，可能导致T细胞功能障碍。此外，T细胞受体β家族的谱系非常有限，这也可能导致无反应。否则，T细胞克隆性增多可能表明存在激活的、可能是自身反应性的T细胞。
