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- Bonfim, C., et al.
(author)
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Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia
- 2016
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In: Biology of Blood and Marrow Transplantation. - : Elsevier BV. - 1083-8791. ; 22:7, s. 1257-1263
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Journal article (peer-reviewed)abstract
- We report on long-term survival in 157 patients with Fanconi anemia (FA) who survived 2 years or longer after their first transplantation with a median follow-up of 9 years. Marrow failure (80%) was the most common indication for transplantation. There were 20 deaths beyond 2 years after transplantation, with 12 of the deaths occurring beyond 5 years after transplantation. Donor chimerism was available for 149 patients: 112 (76%) reported > 95% chimerism, 27 (18%) reported 90% to 95% chimerism, and 8 (5%) reported 20% to 89% donor chimerism. Two patients have < 20% donor chimerism. The 10- and 15-year probabilities of survival were 90% and 79%, respectively. Results of multivariate analysis showed higher mortality risks for transplantations before 2003 (hazard ratio [HR], 7.87; P =.001), chronic graft-versus-host disease (GVHD) (HR, 3.80; P =.004) and squamous cell carcinoma after transplantation (HR, 38.17; P <.0001). The predominant cause of late mortality was squamous cell carcinoma, with an incidence of 8% and 14% at 10 and 15 years after transplantation, respectively, and was more likely to occur in those with chronic GVHD. Other causes of late mortality included chronic GVHD, infection, graft failure, other cancers, and hemorrhage. Although most patients are disease free and functional long term, our data support aggressive surveillance for long periods to identify those at risk for late mortality.
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- Brunstein, Claudio G, et al.
(author)
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Effect of Conditioning Regimen Dose Reduction in Obese Patients Undergoing Autologous Hematopoietic Cell Transplantation
- 2019
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In: Biology of blood and marrow transplantation. - : Elsevier BV. - 1083-8791 .- 1523-6536. ; 25:3, s. 480-487
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Journal article (peer-reviewed)abstract
- Data are limited on whether to adjust high-dose chemotherapy before autologous hematopoietic cell transplant (autoHCT) in obese patients. This study explores the effects of dose adjustment on the outcomes of obese patients, defined as body mass index (BMI) ≥ 30 kg/m2. Dose adjustment was defined as a reduction in standard dosing ≥ 20%, based on ideal, reported dosing and actual weights. We included 2 groups of US patients who had received autoHCT between 2008 and 2014. Specifically, we included patients with multiple myeloma (MM, n = 1696) treated with high-dose melphalan and patients with Hodgkin or non-Hodgkin lymphomas (n = 781) who received carmustine, etoposide, cytarabine, and melphalan conditioning. Chemotherapy dose was adjusted in 1324 patients (78%) with MM and 608 patients (78%) with lymphoma. Age, sex, BMI, race, performance score, comorbidity index, and disease features (stage at diagnosis, disease status, and time to transplant) were similar between dose groups. In multivariate analyses for MM, adjusting for melphalan dose and for center effect had no impact on overall survival (P = .894) and treatment-related mortality (TRM) (P = .62), progression (P = .12), and progression-free survival (PFS; P = .178). In multivariate analyses for lymphoma, adjusting chemotherapy doses did not affect survival (P = .176), TRM (P = .802), relapse (P = .633), or PFS (P = .812). No center effect was observed in lymphoma. This study demonstrates that adjusting chemotherapy dose before autoHCT in obese patients with MM and lymphoma does not influence mortality. These results do not support adjusting chemotherapy dose in this population.
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- Holter-Chakrabarty, Jennifer L., et al.
(author)
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The Sequence of Cyclophosphamide and Myeloablative Total Body Irradiation in Hematopoietic Cell Transplantation for Patients with Acute Leukemia
- 2015
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In: Biology of blood and marrow transplantation. - : Elsevier BV. - 1083-8791 .- 1523-6536. ; 21:7, s. 1251-1257
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Journal article (peer-reviewed)abstract
- Limited clinical data are available to assess whether the sequencing of cyclophosphamide (Cy) and total body irradiation (TBI) changes outcomes. We evaluated the sequence in 1769 (CyTBI, n = 948; TBICy, n = 821) recipients of related or unrelated hematopoietic cell transplantation who received TBI (1200 to 1500 cGY) for acute leukemia from 2003 to 2010. The 2 cohorts were comparable for median age, performance score, type of leukemia, first complete remission, Philadelphia chromosome positive acute lymphoblastic leukemia, HLA-matched siblings, stem cell source, antithymocyte globulin use, TBI dose, and type of graft-versus-host disease (GVHD) prophylaxis. The sequence of TBI did not significantly affect transplantation-related mortality (24% versus 23% at 3 years, P = .67; relative risk, 1.01; P = .91), leukemia relapse (27% versus 29% at 3 years, P = .34; relative risk,.89, P = .18), leukemia-free survival (49% versus 48% at 3 years, P = .27; relative risk,.93; P = .29), chronic GVHD (45% versus 47% at 1 year, P = .39; relative risk,.9; P = .11), or overall survival (53% versus 52% at 3 years, P = .62; relative risk,.96; P = .57) for CyTBI and TBICy, respectively. Corresponding cumulative incidences of sinusoidal obstruction syndrome were 4% and 6% at 100 days (P = .08), respectively. This study demonstrates that the sequence of Cy and TBI does not impact transplantation outcomes and complications in patients with acute leukemia undergoing hematopoietic cell transplantation with myeloablative conditioning.
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- Olsson, R. F., et al.
(author)
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Primary graft failure after myeloablative allogeneic hematopoietic cell transplantation for hematologic malignancies
- 2015
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In: Leukemia. - : Springer Science and Business Media LLC. - 0887-6924 .- 1476-5551. ; 29:8, s. 1754-1762
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Journal article (peer-reviewed)abstract
- Clinical outcomes after primary graft failure (PGF) remain poor. Here we present a large retrospective analysis (n = 23 272) which investigates means to prevent PGF and early detection of patients at high risk. In patients with hematologic malignancies, who underwent their first myeloablative allogeneic hematopoietic cell transplantation, PGF was reported in 1278 (5.5%), and there was a marked difference in PGFs using peripheral blood stem cell compared with bone marrow grafts (2.5 vs 7.3%; P<0.001). A fourfold increase of PGF was observed in myeloproliferative disorders compared with acute leukemia (P<0.001). Other risk factors for PGF included recipient age <30, HLA mismatch, male recipients of female donor grafts, ABO incompatibility, busulfan/cyclophosphamide conditioning and cryopreservation. In bone marrow transplants, total nucleated cell doses <= 2.4 x 10(8) per kg were associated with PGF (odds ratio 1.39; P<0.001). The use of tacrolimus-based immunosuppression and granulocyte colony-stimulating factor were associated with decreased PGF risk. These data, allow clinicians to do more informed choices with respect to graft source, donor selection, conditioning and immunosuppressive regimens to reduce the risk of PGF. Moreover, a novel risk score determined on day 21 post transplant may provide the rationale for an early request for additional hematopoietic stem cells.
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- Pasquini, Marcelo C., et al.
(author)
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Hematopoietic Cell Transplantation Outcomes in Monosomal Karyotype Myeloid Malignancies
- 2016
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In: Biology of blood and marrow transplantation. - : Elsevier BV. - 1083-8791 .- 1523-6536. ; 22:2, s. 248-257
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Journal article (peer-reviewed)abstract
- The presence of monosomal karyotype (MK+) in acute myeloid leukemia (AML) is associated with dismal outcomes. We evaluated the impact of MK+ in AML (MK+AML, n = 240) and in myelodysplastic syndrome (MDS) (MK+MDS, n = 221) on hematopoietic cell transplantation outcomes compared with other cytogenetically defined groups (AML, n = 3360; MDS, n = 1373) as reported to the Center for International Blood and Marrow Transplant Research from 1998 to 2011. MK+AML was associated with higher disease relapse (hazard ratio, 1.98; P < .01), similar transplantation-related mortality (TRM) (hazard ratio, 1.01; P = .90), and worse survival (hazard ratio, 1.67; P < .01) compared with those outcomes for other cytogenetically defined AML. Among patients with MDS, MK+ MDS was associated with higher disease relapse (hazard ratio, 2.39; P < .01), higher TRM (hazard ratio, 1.80; P < .01), and worse survival (HR, 2.02; P < .01). Subset analyses comparing chromosome 7 abnormalities (del7/7q) with or without MK+ demonstrated higher mortality for MK+ disease in for both AML (hazard ratio, 1.72; P < .01) and MDS (hazard ratio, 1.79; P < .01). The strong negative impact of MK+ in myeloid malignancies was observed in all age groups and using either myeloablative or reduced-intensity conditioning regimens. Alternative approaches to mitigate disease relapse in this population are needed.
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- Smiljanic, R., et al.
(author)
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The Gaia-ESO Survey : properties of newly discovered Li-rich giants
- 2018
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In: Astronomy and Astrophysics. - : EDP Sciences. - 0004-6361 .- 1432-0746. ; 617
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Journal article (peer-reviewed)abstract
- Aims. We report 20 new lithium-rich giants discovered within the Gaia-ESO Survey, including the first Li-rich giant with an evolutionary stage confirmed by CoRoT (Convection, Rotation and planetary Transits) data. We present a detailed overview of the properties of these 20 stars. Methods. Atmospheric parameters and abundances were derived in model atmosphere analyses using medium-resolution GIRAFFE or high-resolution UVES (Ultraviolet and Visual Echelle Spectrograph) spectra. These results are part of the fifth internal data release of the Gaia-ESO Survey. The Li abundances were corrected for non-local thermodynamical equilibrium effects. Other stellar properties were investigated for additional peculiarities (the core of strong lines for signs of magnetic activity, infrared magnitudes, rotational velocities, chemical abundances, and Galactic velocities). We used Gaia DR2 parallaxes to estimate distances and luminosities. Results. The giants have A(Li) > 2.2 dex. The majority of them (14 of 20 stars) are in the CoRoT fields. Four giants are located in the field of three open clusters, but are not members. Two giants were observed in fields towards the Galactic bulge, but likely lie in the inner disc. One of the bulge field giants is super Li-rich with A(Li) = 4.0 dex. Conclusions. We identified one giant with infrared excess at 22 mu m. Two other giants, with large v sin i, might be Li-rich because of planet engulfment. Another giant is found to be barium enhanced and thus could have accreted material from a former asymptotic giant branch companion. Otherwise, in addition to the Li enrichment, the evolutionary stages are the only other connection between these new Li-rich giants. The CoRoT data confirm that one Li-rich giant is at the core-He burning stage. The other giants are concentrated in close proximity to the red giant branch luminosity bump, the core-He burning stages, or the early-asymptotic giant branch. This is very clear from the Gaia-based luminosities of the Li-rich giants. This is also seen when the CoRoT Li-rich giants are compared to a larger sample of 2252 giants observed in the CoRoT fields by the Gaia-ESO Survey, which are distributed throughout the red giant branch in the T-eff-log g diagram. These observations show that the evolutionary stage is a major factor for the Li enrichment in giants. Other processes, such as planet accretion, contribute at a smaller scale.
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- Fernandes, Juliana Folloni, et al.
(author)
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Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries.
- 2018
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In: Journal of clinical immunology. - : Springer Science and Business Media LLC. - 1573-2592 .- 0271-9142. ; 38:8, s. 917-926
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Journal article (peer-reviewed)abstract
- The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n=123). The median age at HSCT was 22months, and the most common diseases were severe combined immunodeficiency (SCID) (n=67) and Wiskott-Aldrich syndrome (WAS) (n=67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n=53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients' conditions at the moment of transplant.
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- Hasan, M., et al.
(author)
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Robust superhydrophobic cellulose nanofiber aerogel for multifunctional environmental applications
- 2019
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In: Polymers. - : MDPI AG. - 2073-4360. ; 11:3
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Journal article (peer-reviewed)abstract
- The fabrication of superadsorbent for dye adsorption is a hot research area at present. However, the development of low-cost and highly efficient superadsorbents against toxic textile dyes is still a big challenge. Here, we fabricated hydrophobic cellulose nanofiber aerogels from cellulose nanofibers through an eco-friendly silanization reaction in liquid phase, which is an extremely efficient, rapid, cheap, and environmentally friendly procedure. Moreover, the demonstrated eco-friendly silanization technique is easy to commercialize at the industrial level. Most of the works that have reported on the hydrophobic cellulose nanofiber aerogels explored their use for the elimination of oil from water. The key novelty of the present work is that the demonstrated hydrophobic cellulose nanofibers aerogels could serve as superadsorbents against toxic textile dyes such as crystal violet dye from water and insulating materials for building applications. Here, we make use of the possible hydrophobic interactions between silane-modified cellulose nanofiber aerogel and crystal violet dye for the removal of the crystal violet dye from water. With a 10 mg/L of crystal violet (CV) aqueous solution, the silane-modified cellulose nanofiber aerogel showed a high adsorption capacity value of 150 mg/g of the aerogel. The reason for this adsorption value was due to the short-range hydrophobic interaction between the silane-modified cellulose nanofiber aerogel and the hydrophobic domains in crystal violet dye molecules. Additionally, the fabricated silane-modified cellulose nanofiber hydrophobic aerogels exhibited a lower thermal conductivity value of 0.037 W·m -1 K -1 , which was comparable to and lower than the commercial insulators such as mineral wools (0.040 W·m -1 K -1 ) and polystyrene foams (0.035 W·m -1 K -1 ). We firmly believe that the demonstrated silane-modified cellulose nanofiber aerogel could yield an eco-friendly adsorbent that is agreeable to adsorbing toxic crystal violet dyes from water as well as active building thermal insulators.
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- Smiljanic, R., et al.
(author)
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The Gaia-ESO Survey : Inhibited extra mixing in two giants of the open cluster Trumpler 20?
- 2016
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In: Astronomy and Astrophysics. - : EDP Sciences. - 0004-6361 .- 1432-0746. ; 591
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Journal article (peer-reviewed)abstract
- We report the discovery of two Li-rich giants, with A(Li) ~ 1.50, in an analysis of a sample of 40 giants of the open cluster Trumpler 20 (with turnoff mass ~1.8 M⊙). The cluster was observed in the context of the Gaia-ESO Survey. Methods. The atmospheric parameters and Li abundances were derived using high-resolution UVES spectra. The Li abundances were corrected for nonlocal thermodynamical equilibrium (non-LTE) effects. Results. Only upper limits of the Li abundance could be determined for the majority of the sample. Two giants with detected Li turned out to be Li rich: star MG 340 has A(Li)non-LTE = 1.54 ± 0.21 dex and star MG 591 has A(Li)non-LTE = 1.60 ± 0.21 dex. Star MG 340 is on average ~0.30 dex more rich in Li than stars of similar temperature, while for star MG 591 this difference is on average ~0.80 dex. Carbon and nitrogen abundances indicate that all stars in the sample have completed the first dredge-up. Conclusions. The Li abundances in this unique sample of 40 giants in one open cluster clearly show that extra mixing is the norm in this mass range. Giants with Li abundances in agreement with the predictions of standard models are the exception. To explain the two Li-rich giants, we suggest that all events of extra mixing have been inhibited. This includes rotation-induced mixing during the main sequence and the extra mixing at the red giant branch luminosity bump. Such inhibition has been suggested in the literature to occur because of fossil magnetic fields in red giants that are descendants of main-sequence Ap-type stars.
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