Bone Marrow Transplantation Center

İzmit, Turkey

Bone Marrow Transplantation Center

İzmit, Turkey
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Teke H.U.,Eskiehir Osmangazi University | Gunduz E.,Eskiehir Osmangazi University | Akay O.M.,Eskiehir Osmangazi University | Gulbas Z.,Bone Marrow Transplantation Center
Platelets | Year: 2013

Primer immunologic defect in patients with idiopathic thrombocytopaenic purpura (ITP) result from autoreactive B-lymphocytes secreting antiplatelet antibodies. Dysfunctional cellular immunity has also great importance in ITP pathogenesis. CD4+CD25+ regulatory T-cells have immunoregulatory features and it is able to inhibit CD4+CD25 - and CD8+ responses. ITP is also an autoimmune disease; the CD4+CD25+ T-cell levels of the patients decrease during the active state. According to our findings, immunosuppressive treatments increase the CD4+CD25+ Treg cell levels in the non-remission ITP patients. However, this level is not enough to overcome the resistance. CD4+CD25-Foxp3+ and CD4 +Foxp3+ Treg cells are responsible for the pathogenesis of the non-remission ITP patients and other factors exist, which are responsible for the resistance of ITP treatment. © 2013 Informa UK Ltd.

Sahin D.G.,Eskiehir Osmangazi University | Gunduz E.,Eskiehir Osmangazi University | Kasifoglu N.,Eskiehir Osmangazi University | Akay O.M.,Eskiehir Osmangazi University | And 2 more authors.
Advances in Therapy | Year: 2013

Introduction: Cytomegalovirus (CMV) infections continue to cause significant morbidity and mortality in hematopoietic stem cell transplant (HSCT) recipients. Successful pre-emptive therapy in transplant patients depends on the availability of reliable diagnostic tests for CMV infections. The purpose of this retrospective study was to evaluate CMV DNA viral load, incidence of CMV disease and CMV seropositivity, risk factors and correlation between CMV DNA positivity and clinical course in HSCT patients. Methods: Two hundred and twenty-five patients who underwent peripheral blood stem cell or bone marrow transplantation between June 2003 and April 2010 were included. A realtime polymerase chain reaction (RT-PCR) assay was used for CMV monitoring. Results: Recipient median age was 42.5 years. CMV seropositivity was 95.6%. CMV DNA positivity determined by RT-PCR was 24.9% among the entire patient group. CMV DNA positivity with RT-PCR was found to be significantly higher in allogeneic transplant recipients than autologous transplant recipients (46.7% vs 14.0%; P<0.0001). Gender, age, conditioning regimen, stem cell source, underlying disease and recipient and donor seropositivity (alone or paired) were not significant risk factors for CMV DNAemia. We did not observe any CMV end-organ disease. Conclusion: CMV DNAemia was significantly higher in allogeneic transplant recipients than in autologous transplant patients. End-organ disease could be prevented with appropriate pre-emptive therapy. © Springer Healthcare 2013.

PubMed | Abderahman Mami Hospital, Mongi Slim Hospital, Bone Marrow Transplantation Center, Habib Bourguiba Hospital and 4 more.
Type: Journal Article | Journal: Immunogenetics | Year: 2016

Immunoglobulin class switch recombination deficiencies (Ig-CSR-D) are characterized by normal or elevated serum IgM level and absence of IgG, IgA, and IgE. Most reported cases are due to X-linked CD40L deficiency. Activation-induced cytidine deaminase deficiency is the most frequent autosomal recessive form, whereas CD40 deficiency is more rare. Herein, we present the first North African study on hyper IgM (HIGM) syndrome including 16 Tunisian patients. Phenotypic and genetic studies allowed us to determine their molecular basis. Three CD40LG mutations have been identified including two novels (c.348_351dup and c.782_*2del) and one already reported mutation (g.6182G>A). No mutation has been found in another patient despite the lack of CD40L expression. Interestingly, three AICDA mutations have been identified in 11 patients. Two mutations were novel (c.91T>C and c.389A>C found in one and five patients respectively), and one previously reported splicing mutation (c.156+1T>G) was found in five patients. Only one CD40-deficient patient, bearing a novel mutation (c.109T>G), has been identified. Thus, unlike previous reports, AID deficiency is the most frequent underlying molecular basis (68%) of Ig-CSR-D in Tunisian patients. This finding and the presence of specific recurrent mutations are probably due to the critical role played by inbreeding in North African populations.

PubMed | Nuclear Medicine Unit, Bone Marrow Transplantation Center, University of Würzburg, University of Milan and 5 more.
Type: Journal Article | Journal: Journal of translational medicine | Year: 2016

The trophic, anti-apoptotic and regenerative effects of bone marrow mesenchymal stromal cells (MSC) may reduce neuronal cell loss in neurodegenerative disorders.We used MSC as a novel candidate therapeutic tool in a pilot phase-I study for patients affected by progressive supranuclear palsy (PSP), a rare, severe and no-option form of Parkinsonism. Five patients received the cells by infusion into the cerebral arteries. Effects were assessed using the best available motor function rating scales (UPDRS, Hoehn and Yahr, PSP rating scale), as well as neuropsychological assessments, gait analysis and brain imaging before and after cell administration.One year after cell infusion, all treated patients were alive, except one, who died 9months after the infusion for reasons not related to cell administration or to disease progression (accidental fall). In all treated patients motor function rating scales remained stable for at least six-months during the one-year follow-up.We have demonstrated for the first time that MSC administration is feasible in subjects with PSP. In these patients, in whom deterioration of motor function is invariably rapid, we recorded clinical stabilization for at least 6months. These encouraging results pave the way to the next randomized, placebo-controlled phase-II study that will definitively provide information on the efficacy of this innovative approach. Trial registration NCT01824121.

Arora M.,University of Minnesota | Klein J.P.,Medical College of Wisconsin | Weisdorf D.J.,University of Minnesota | Hassebroek A.,Center for International Blood and Marrow Transplant Research | And 21 more authors.
Blood | Year: 2011

Several risk factors are associated with increased mortality in patients with chronic graft-versus-host disease (cGVHD), but there is considerable variability in the reported factors. Therefore, we evaluated patient, transplantation, and cGVHD characteristics to develop a risk score in 5343 patients with cGVHD. Ten variables were identified as being significant in multivariate analysis of overall survival and nonrelapse mortality (NRM): age, prior acute GVHD, time from transplantation to cGVHD, donor type, disease status at transplantation, GVHD prophylaxis, gender mismatch, serum bilirubin, Karnofsky score, and platelet count. These 10 variables were used to build a cGVHD risk score, and 6 risk groups (RGs) were identified. The 5-year NRM was 5% (1%-9%) in RG1, 20% (19%-23%) in RG2, 33% (29%-37%) in RG3, 43% (40%-46%) in RG4, 63% (53%-74%) in RG5, and 72% (59%-85%) in RG6. The 5-year overall survival was highest at 91% (95% confidence interval [CI]:85%-97%) in RG1, followed by 67% (65%-69%) in RG2, 51% (46%-55%) in RG3, 40% (37%-43%) in RG4, 21% (12%-30%) in RG5, and 4% (0%-9%) in RG6 (all P < .01). This analysis demonstrates the usefulness of data from a large registry to develop risk-score categories for major transplantation outcomes. Validation of this cGVHD risk score is needed in a different population to ensure its broad applicability. © 2011 by The American Society of Hematology.

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