Cancers, Vol. 12, Pages 597: mTOR and STAT3 Pathway Hyper-Activation is Associated with evated Interleukin-6 Levels in Patients with Shwachman-Diamond Syndrome: Further Evidence of Lymphoid Lineage Impairment

Cancers, Vol. 12, Pages 597: mTOR and STAT3 Pathway Hyper-Activation is Associated with evated Interleukin-6 Levels in Patients with Shwachman-Diamond Syndrome: Further Evidence of Lymphoid Lineage Impairment Cancers doi: 10.3390/cancers12030597 Authors: Antonio Vella Elisabetta D’Aversa Martina Api Giulia Breveglieri Marisole Allegri Alice Giacomazzi Elena Marinelli Busilacchi Benedetta Fabrizzi Tiziana Cestari Claudio Sorio Gloria Bedini Giovanna D’Amico Vincenzo Bronte Antonella Poloni Antonio Benedetti Chiara Bovo Seth J. Corey Monica Borgatti Marco Cipolli Valentino Bezzerri Shwachman–Diamond syndrome (SDS) is a rare inherited bone marrow failure syndrome, resulting in neutropenia and a risk of myeloid neoplasia. A mutation in a ribosome maturation factor accounts for almost all of the cases. Lymphoid involvement in SDS has not been well characterized. We recently reported that lymphocyte subpopulations are reduced in SDS patients. We have also shown that the mTOR-STAT3 pathway is hyper-activated in SDS myeloid cell populations. Here we show that mTOR-STAT3 signaling is markedly upregulated in the lymphoid compartment of SDS patients. Furthermore, our data reveal elevated IL-6 levels in cellular supernatants obtained from lymphoblasts, bone marrow mononuclear and mesenchymal stromal cells, and plasma samples obtained from a cohort of 10 patients. Of note, everolimus-mediated inhibition of mTOR signaling is associated wi...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Article Source Type: research