Table 6.

MPS VI with HSCT

Author	Details	Results
Turbeville et al. 2011 [17]	Between 1982–2007 45 patients Most common regimen: cyclophosphamide and busulfan Most common donor source bone marrow from unrelated donor	85% survival rate six months after transplantation 56% alive and engrafted rate six months after transplantation 34 patients developed acute GvHD
Behfar et al. 2017 [62,165]	3 severe MPS VI patients Non-sibling donors	Two of the patients achieved 95% chimerism Patient 1: Received BMT Donor cells from carrier mother Increased enzyme levels Improved joint mobility and ability to walk and climb Improved pulmonary function Decreased overall facial coarseness and finger stiffness Corneal clouding remained unchanged Patient 2: Received a PBSCT Grandmother donor Developed acute GVHD grade II Normal spleen size and liver size Improved walk test Mild tricuspid regurgitation and mitral regurgitation remained Patient 3: Unrelated cord blood donor 5/6 HLA-matched Experienced graft failure Died of pneumonia 11 months after transplantation