The clinico-immunological surveillance of neurofibromatosis

• Published in: Minerva medica Vol. 85; no. 5; p. 221
• Main Authors: Gerosa, P, Vai, C, Canepari, C, Fontana, A, Bizzozero, L, Giussani, G, Spinelli, M
• Format: Journal Article
• Language: Italian
• Published: Italy 01-05-1994
• Subjects: Adolescent; Adult; Biomarkers - blood; Child; Diseases in Twins; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neurofibromatosis 1 - epidemiology; Neurofibromatosis 1 - immunology; Population Surveillance; Twins, Dizygotic
• ISSN: 0026-4806

Considering the more recent physiopathogenetic advancements in neurofibromatosis (NF), we propose to employ novel instrumental and laboratory procedures for the immunological and clinical surveillance of NF. In NF the evolution of the non-self can lead to disease expansion, at times transforming into malignancy. Contemporarily, the resulting immunological reactivity can either lead to the type of fibrosis that one sees in paraneoplastic connective tissue disease or be deficient. Through interdisciplinary biohumoral analyses were carried out contemporarily so as to gain comparative insight into the eventual unfolding of immunological and fibrotic phenomena. The range of the clinical follow-up varied from one to four years with periodic day hospital admissions. We studied ten NF patients that originated from southern Italy and belonged to middle and lower middle class status; we also studied one healthy subject who had the same HLA haplotype as his NF affected twin. We performed biohumoral analyses in clinical stable patients and saw moderate variations in induces useful for monitoring the evolution of this dysplastic-neoplastic condition, e.g. procollagens, interleukin-2, NSE (assayed with radioimmunological methods) and complement these parameters proved to be of use in monitoring the fibrotic evolution of NF. The intent of our work was to complete the earlier studies on NF surveillance, especially during periods of disease evolution and immuno-fibrotic alteration.