Abstract < /h3 > < p class= " a-plus-plus " > Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome caused by mutations of the < em class= " a-plus-plus " > NF1 < /em > gene and resulting dysregulation of the Ras-pathway. In addition to peripheral nerve tumors, affected tissues include the musculoskeletal and cardiovascular system. The immune system has recen..."> Abstract < /h3 > < p class= " a-plus-plus " > Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome caused by mutations of the < em class= " a-plus-plus " > NF1 < /em > gene and resulting dysregulation of the Ras-pathway. In addition to peripheral nerve tumors, affected tissues include the musculoskeletal and cardiovascular system. The immune system has recen..." /> Abstract < /h3 > < p class= " a-plus-plus " > Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome caused by mutations of the < em class= " a-plus-plus " > NF1 < /em > gene and resulting dysregulation of the Ras-pathway. In addition to peripheral nerve tumors, affected tissues include the musculoskeletal and cardiovascular system. The immune system has recen..." />

Effector T cell subclasses associate with tumor burden in neurofibromatosis type 1 patients

< h3 class= " a-plus-plus " > Abstract < /h3 > < p class= " a-plus-plus " > Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome caused by mutations of the < em class= " a-plus-plus " > NF1 < /em > gene and resulting dysregulation of the Ras-pathway. In addition to peripheral nerve tumors, affected tissues include the musculoskeletal and cardiovascular system. The immune system has recently been suggested as a possible modulator NF1-related phenotypes. Therefore, we determined the immune phenotype in NF1 patients and investigated its relationship with the phenotypic severity of NF1-related tumor manifestations. We quantified global leukocytes and lymphocyte subpopulations of peripheral blood from 37 NF1 patients and 21 healthy controls by flow cytometry. To associate immune phenotype with tumor phenotype, all NF1 patients underwent whole-body magnetic resonance imaging and total internal tumor volume was calculated. The immunophenotypes were compared among four NF1 groups with different total internal tumor burdens and between NF1 patients and non-NF1 subjects. We found that NF1 patients show a generalized lymphopenia. Closer analysis revealed that the CD8 < sup class= " a-plus-plus " > + < /sup > /CD27 < sup class= " a-plus-plus " > − < /sup > and CD8 < sup class= " a-plus-plus " > + < /sup > /CD57 < sup class= " a-plus-plus " > + < /sup > effector T cell fractions strongly increase in NF1 patients with low tumor load and decrease to levels below control in pat...
Source: Cancer Immunology, Immunotherapy - Category: Cancer & Oncology Source Type: research