The Bonfils Blood Center

Denver, CO, United States

The Bonfils Blood Center

Denver, CO, United States

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Alsultan A.,University of Colorado at Denver | Sokol R.J.,Clinical Translational Science Institute | Lovell M.A.,University of Colorado at Denver | Thurman G.,University of Colorado at Denver | Ambruso D.R.,The Bonfils Blood Center
Pediatric Blood and Cancer | Year: 2010

We present a 23-year-old female with Glycogen storage disease Ib (GSD Ib) who was diagnosed with ulcerative colitis-like inflammatory bowel disease (IBD) at 7 years of age. G-CSF therapy reversed the IBD, was required to maintain IBD remission and was well tolerated. Neutrophil functions at time of diagnosis showed impaired chemotaxis but normal superoxide anion production and bactericidal activity. Ulcerative colitis-like IBD may also be seen in GSD Ib and is responsive to G-CSF therapy. Neutrophil dysfunction is variable among patients with GSD Ib. © 2010 Wiley-Liss, Inc.


PubMed | The Bonfils Blood Center
Type: Journal Article | Journal: Vox sanguinis | Year: 2010

Mirasol Pathogen Reduction Technology (PRT) treatment uses riboflavin and UV light to inactivate pathogens in blood components. Neutrophil [polymorphonuclear cells (PMN)] priming activity accumulates during routine storage of cellular blood components, and this activity has been implicated in transfusion-related acute lung injury (TRALI). We hypothesize that PRT-treatment of blood components affects the priming activity generated during storage of packed RBCs (PRBCs) or platelet concentrates (PCs), which can elicit ALI in vivo.Plasma, PRBCs and PCs were isolated from healthy donors whole blood or by apheresis. Half of a collected unit was treated with PRT treatment and the remainder was left as an unmodified control. Supernatant was collected during storage of PCs and PRBCs and assayed for PMN priming activity and used as the second event in a two-event in vivo model of TRALI.PRT treatment did not induce priming activity in plasma or affect the priming activity generated during storage of PCs or PRBCs as compared with the unmodified controls. The supernatants from stored, but not fresh, PCs and PRBCs did cause ALI as the second event in a two-event animal model of TRALI, which was unaffected by PRT treatment. We conclude that the PRT treatment does not induce priming activity in plasma nor does it affect the priming activity generated during storage of PCs or PRBCs or their ability to cause ALI as the second event in a two-event in vivo model of TRALI. Moreover, the amount of priming activity in TRIMA-isolated PCs was significantly less than SPECTRA-isolated PCs.

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