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Long-Term Outcome and Evaluation of Organ Function in Pediatric Patients Undergoing Haploidentical and Matched Related Hematopoietic Cell Transplantation for Sickle Cell Disease
We report the long-term outcomes of 22 pediatric patients who underwent related donor HSCT for SCD at St. Jude Children's Research Hospital, either a myeloablative sibling MRD HSCT (n = 14) or reduced-intensity parental haploidentical donor HSCT (n = 8). The median patient age was 11.0 ± 3.9 years in the MRD graft recipients and 9.0 ± 5.0 years in the haploidentical donor graft recipients. The median follow-up was 9.0 ± 2.3 years, with an overall survival (OS) of 93% and a recurrence/graft failure rate of 0%, for the MRD cohort and 7.4 ± 2.4 years, with an OS of 75%, disease-free survival of 38%, and disease recurren...
Source: Biology of Blood and Marrow Transplantation - February 18, 2013 Category: Hematology Authors: Mari H. Dallas, Brandon Triplett, David R. Shook, Christine Hartford, Ashok Srinivasan, Joseph Laver, Russell Ware, Wing Leung Tags: Clinical Research Source Type: research

A Polymorphism in Toll-like Receptor 2 Gene Is Associated with Occurrence of Bacterial Infections in Sickle Cell Disease Patients
Discussion: In SCD pts, TLR-2 rs4696480 TA genotype might be protective against bacterial infections, whereas TT genotype might increase risk of such infections. Previous reports demonstrated higher secretion of inflammatory factors in cells from AA individuals, lower occurrence and severity of immune diseases in T carriers. TA genotype might stand between deleterious effects of over inflammatory response (AA genotype) and under response (TT genotype) to infectious agents.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Tozatto-Maio, K., Girot, R., Ly, I. D., Rocha, V., Pinto, A. C. S., Diagne, I., Benzerara, Y., Dinardo, C. L., Kashima, S., Araujo, I. L., Kenzey, C., Fonseca, G. H. H., Rodrigues, E., Volt, F., Jarduli, L. R., Ruggeri, A., Mariaselvam, C. M., Gualandro, Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research