T-cell dysfunction in hairy cell leukemia: an updated review.
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.
Department of Hematology, Laboratory of Experimental Hematology, Leiden University Medical Center, The Netherlands.
L
L
Van De Corput
J H
JH
Falkenburg
J C
JC
Kluin-Nelemans
D002448:Cell Adhesion / Q000276:immunology; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000276:immunology*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000276:immunology*; D008213:Lymphocyte Activation; D008228:Lymphoma, Non-Hodgkin / Q000276:immunology*; D011948:Receptors, Antigen, T-Cell / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology*
D011948:Receptors, Antigen, T-Cell