Management of Thymectomised Myasthenic Patients

Nix, Wilfred A.; Große-Höötmann, H.; Kirchner, Thomas; Marx, Alexander

doi:10.1007/978-1-4899-0033-3_47

Cited by 1 publication

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“…However, other lymphoid organs and probably the bone marrow also have to play a role in this process, because extended thymomectomy (including the residual thymus) is not followed by a decline but by an increase of autoantibody titers instead (Fig. 4a) (Somnier, 1994;Nix et al, 1997;Schalke et al, 1997). (b) Increased frequency of AChR and neurofilament-reactive T cells in the peripheral blood week after surgery in MG patients with mixed and cortical thymoma (n 9).…”

Section: Introductionmentioning

confidence: 99%

Paraneoplastic Autoimmunity in Thymus Tumors

Marx

Schultz

Wilisch

et al. 1997

Journal of Immunology Research

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Autoimmune phenomena are more frequent in thymic epithelial tumors (TET) than in any other human tumor. Mysthenia gravis (MG) is by far the most common autoimmune disease in thymoma patients. MG is characterized by muscle weakness due to autoantibodies against the acetylcholine receptor (AChR), and CD4 AChR-specific T cells play a pivotal role for the production of these autoantibodies. About 10% of MG patients have a thymoma and, interestingly, only such thymomas exhibit an MG association that maintains thymuslike morphological and functional features with respect to the homing and differentiation of immature T cells. Since AChR protein is not expressed in thymomas, the specificity of the autoimmunity in thymoma-associated MG is thought to be determined by nonreceptor proteins with AChR epitopes. Such proteins are overexpressed in cortical-type MG-associated thymomas, and medullary thymomas express these proteins at barely detectable levels. Aside from this quantitative difference, the pathogenesis of anti-AChR autoimmunity might be qualitatively different in these thymoma subtypes. Our findings suggest that an antigen-specific abnormal Tcell selection,by cortical-type TET may contribute to the pathogenesis of paraneoplastic MG. In contrast, an abnormal (intratumorous) activation of autoreactive T cells may be operative in medullary thymomas.

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Section: Introductionmentioning

confidence: 99%