TY - JOUR A1 - Kuçi, Zyrafete A1 - Jordan, Christiane A1 - Wehner, Sibylle A1 - Sörensen, Jan A1 - Jarisch, Andrea A1 - Salzmann-Manrique, Emilia A1 - Pfeffermann, Lisa-Marie A1 - Klingebiel, Thomas A1 - Bader, Peter A1 - Kuçi, Selim T1 - The phenotype and functional activity of mesenchymal stromal cells in pediatric patients with non-malignant hematological disease T2 - Cells N2 - As the biology of mesenchymal stromal cells (MSCs) in patients with non-malignant hematological diseases (NMHD) is poorly understood, in the current study we performed a basic characterization of the phenotype and functional activity of NMHD-MSCs. Bone marrow (BM) of patients with thalassemia major (TM) possessed a significantly higher number of nucleated cells (BM-MNCs)/mL BM than healthy donors (P < 0.0001), which however did not result in a higher number of colony forming units-fibroblast (CFU-F) per milliliter BM. In contrast, from 1 × 106 BM-MNCs of patients with sickle cell disease (SCD) were generated significantly more CFU-Fs than from TM-BM-MNCs (P < 0.013) and control group (P < 0.02). In addition, NMHD-MSCs expressed significantly lower levels of CD146 molecule, demonstrated an equal proliferation potential and differentiated along three lineages (osteoblasts, chondrocytes and adipocytes) as healthy donors’ MSCs, with exception of TM-MSCs which differentiated weakly in adipocytes. In contrast to other NMHD-MSCs and healthy donors’ MSCs, TM-MSCs demonstrated an impaired in vitro immunosuppressive potential, either. Noteworthy, the majority of the immunosuppressive effect of NMHD-MSCs was mediated through prostaglandin-E2 (PGE2), because indomethacin (an inhibitor of PGE2 synthesis) was able to significantly reverse this effect. Our results indicate therefore that NMHD-MSCs, except TM-MSCs, may be used as an autologous cell-based therapy for post-transplant complications such as graft failure, graft-versus-host disease (GvHD) and osteonecrosis. KW - mesenchymal stromal cells KW - non-malignant hematological diseases KW - thalassemia KW - sickle cell anemia KW - severe congenital neutropenia Y1 - 2020 UR - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/54422 UR - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-544229 N1 - This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. VL - 9 IS - 431 PB - MDPI CY - Basel ER -