C. Jeandel et al., THYMOMA WITH IMMUNODEFICIENCY (GOODS-SYNDROME) ASSOCIATED WITH SELECTIVE COBALAMIN MALABSORPTION AND BENIGN IGM-KAPPA GAMMOPATHY, Journal of internal medicine, 235(2), 1994, pp. 179-182
We have described the first case, to our knowledge, in which recurrent
respiratory tract infections were the primary manifestation of thymom
a with immunodeficiency (Good's syndrome) associated with cobalamin ma
labsorption and immunoglobulin M-kappa (IgM-kappa)M component. The int
rinsic factor receptor activity was dramatically decreased in a mucosa
l homogenate prepared from ileal biopsies. This decreased activity cou
ld be the principal cause of the malabsorption of labelled cobalamin w
hich was observed in the presence of intrinsic factor. However, it cou
ld be the consequence of the cobalamin deficiency, as it is known that
a cobalamin deficiency can affect the assimilation of cobalamin, even
in presence of exogenous intrinsic factor.