title: Thymic dysfunction in childhood T-acute lymphoblastic leukemia: a possible linkage with a primary thymus involvement.
abstract: Experimental models, clinical and histopathological observations suggest a thymic origin of childhood T acute lymphoblastic leukemia (T-ALL). We studied thymic epithelial function in childhood T-ALL as compared to normal controls in order to improve our understanding of the cellular immunodeficiency mechanisms operating in a thymus-linked malignant process. The levels of Facteur Thymique Sérique (FTS) were measured in 9 patients at diagnosis, according to the rosette inhibition assay of Dardenne & Bach (1975). This method is based on the capacity of human serum containing FTS activity to confer on rosette-forming cells (RFC) from adult thymectomized mice a sensitivity to azathioprine identical to that of normal mouse RFC. All patients presented low age-corrected titres of FTS. No zinc deficiency was found, suggesting that low FTS levels are not related to unexpressed FTS biological activity. Plasma from all the children studied contained factors capable of inhibiting the biological activity of FTS in vitro. However, the nature of this inhibitor has not yet been elucidated. Our study shows the presence of a thymic dysfunction in childhood T-ALL, which could partially explain the immunodeficiency described in these patients. The linkage of the leukemic process with a primitive thymic involvement is discussed.
authors.affiliation: Istituto di Clinica Pediatrica di Pisa, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Consolini
authors.forename: A
authors.initials: A
authors.lastname: Legitimo
authors.forename: A
authors.initials: A
authors.lastname: Giorgianni
authors.forename: M C
authors.initials: MC
authors.lastname: Putti
mesh_terms: D000293:Adolescent; D000818:Animals; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000097:blood / Q000503:physiopathology*; D008297:Male; D051379:Mice; D014411:Neoplastic Stem Cells / Q000473:pathology; D013935:Thymic Factor, Circulating / Q000172:deficiency*; D013950:Thymus Gland / Q000503:physiopathology*
chemical_list: D013935:Thymic Factor, Circulating