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Dr. Adrian Man
Impact of allogeneic hematopoietic stem cell transplantation on the biological parameters of immunity in patients with chronic granulomatous disease Cristian Jinca, Smaranda Arghirescu, Mihaela Bataneant, Mihaela, Andrada Oprisoni, Anca Isac, Ioana Ionita, Malina Popa, Valentin Ordodi, Margit Serban
Abstract: Donor derived neutrophil reconstitution, defined as the normalization of the neutrophil counts and function after allogeneic hematopoietic stem cell transplantation (HSCT), represents a prerequisite for the cure of patients with chronic granulomatous disease (CGD), expressed by a normalization of the nitroblue tetrazolium assay (NBT test), of the dihydro-rhodamine 123 (DHR) oxidation and of other confirmatory tests. These investigations together with the flowcytometric analysis of the lymphocyte subsets reconstitution at repeated post-transplant intervals predict the outcome after HSCT in these patients. Because a normalization of phagocyte activity implies a complete neutrophil engraftment with the presence of a stable and functional donor-derived neutrophil population in the transplant recipient, lineage-specific chimerism analysis represents also an important follow-up tool. All the above mentioned parameters have been investigated in two patients with CGD with a long history of life-threatening fungal and bacterial infections non-responsive to specific anti-infectious therapies, in whom the HSCT represented the only chance for cure. We showed that the hematological reconstitution in terms of absolute neutrophil count and function was very fast, within 1 month post-transplant, leading to a decisive and immediate clinical improvement of the patients, despite the delayed reconstitution of the lymphocyte subsets. The normalization of the neutrophil function tests in both patients correlated well with the achieved full donor chimerism and with the clinical outcome of these patients after the transplant. Further controlled clinical studies are warranted in order to comparatively assess the reconstitution of the lymphocyte subsets in bone marrow versus peripheral blood stem cell transplantation for CGD Keywords: Analysis of immune reconstitution,hematopoietic chimerism analysis,leukocyte oxidative burst chronic granulomatous disease,hematopoietic stem cell transplantation. |
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Jinca C, Arghirescu S, Bataneant M, Mihaela , Oprisoni A, Isac A, et al. Impact of allogeneic hematopoietic stem cell transplantation on the biological parameters of immunity in patients with chronic granulomatous disease. Rev Romana Med Lab. 2012;20(1):21-31
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