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ABSTRACTAeglais a genus of freshwater anomuran Crustacea known for having epimorphic postembryonic development. The newly-hatched juveniles’ morphology have been described for only 11 of the 93 known species. These descriptions are important tools for identifying juveniles and useful characteristics for phylogenetic analyses. This study aims to describe and illustrate the external morphology of the first juvenile stage of Aegla lata. Ovigerous females were collected, fed, and monitored periodically in the laboratory until the eggs hatched. The newly-hatched juveniles were fixed in an alcohol series and preserved in a 70% alcohol and glycerine solution (2:1 ratio) for subsequent dissection and analysis under light microscopy. The appendages were illustrated using a camera lucida and then described. Scanning electron microscopy was used to analyse some specimens, allowing the detailed observation of the setal morphology, some cephalothoracic appendages, and lineae aeglicae. Pappose setae were also observed in the protopod and dactylus of cheliped. The newly-hatched juvenile of A. latahas a set of the following characteristics: basal segment of antennule with serrate setae; distal segment of antennule with pappose setae; merus of second maxilliped with simple setae; and merus of third maxilliped without simple setae.

The aim of this study was to determine the activity of Brevibacillus brevisstrain LABIM17 against Xanthomonas euvesicatoriapv. euvesicatoriaand the control of bacterial spot on tomato. The antimicrobial activity of the strain LABIM17 against X. euvesicatoriapv. euvesicatoriastrain 75-3 was evaluated by the agar diffusion method, the pour plate. The minimum inhibitory concentration (MIC) for the growth of the strain 75-3 was 500 µg mL−1of the LABIM17 extract, the lyophilized cells free supernatant (CFS). Tomato plants sprayed with LABIM17 suspension at the concentrations of 3.4 × 107and 1.7 × 107CFU mL−1and inoculated three days later with strain 75-3 showed reduction in the severity of the disease. Further, the plants that received these applications of strain LABIM17 presented reduction of 80 and 86%, respectively, in bacterial spot severity, the same level of disease reduction provided by the commercial chemical inducer acibenzolar-S-methyl. To our knowledge, this is the first report of the antimicrobial activity of B. brevisstrain LABIM17 against X. euvesicatoriapv. euvesicatoria. The characterization of this antimicrobial activity may bring new insights on the mechanisms of control of bacterial spot in tomato by this promising biocontrol agent.

Introduction Neoadjuvant endocrine therapy (NET) is recommended for the treatment of invasive breast cancer (BC), particularly luminal subtypes, in locally advanced stages. Previous randomized studies have demonstrated the benefits of aromatase inhibitors in this context. However, NET is typically reserved for elderly or frail patients who may not tolerate neoadjuvant chemotherapy. Identifying non-responsive patients early and extending treatment for responsive ones would be ideal, yet optimal strategies are awaited.Aims This non-randomized phase 2 clinical trial aims to assess NET feasibility and efficacy in postmenopausal stage II and III luminal BC patients, identifying predictive therapeutic response biomarkers. Efficacy will be gauged by patients with Ki67 ≤ 10% after 4 weeks and Preoperative Endocrine Prognostic Index (PEPI) scores 0 post-surgery. Study feasibility will be determined by participation acceptance rate (recruitment rate ≥50%) and inclusion rate (>2 patients/month).Methods Postmenopausal women with luminal, HER2-tumors in stages II and III undergo neoadjuvant anastrozole treatment, evaluating continuing NET or receiving chemotherapy through early Ki67 analysis after 2 to 4 weeks. The study assesses NET extension for up to 10 months, using serial follow-ups with standardized breast ultrasound and clinical criteria-based NET suspension. Clinical and pathological responses will be measured overall and in the luminal tumor A subgroup. Toxicity, health-related quality of life, and circulating biomarkers predicting early NET response will also be evaluated.

•CD276 (B7-H3)gene expression is associated with BCG unresponseviness in NMIBC patients.•IDO1expression is a marker of worse prognosis in NMIBC.•MSI and PD-L1 expressions are not associated with responsiveness of BCG vaccine in NMIBC patients.

Prophylaxis for GVHD is an evolutionary scenario, with new drugs and approaches being developed. Therefore, it is important to know if these changes are incorporated into clinical practice particularly in low- or middle-income countries.

Therapy for acute GVHD are evolutionary scenarios, with new drugs being developed. Therefore, it is important to know how fast these changes are incorporated into clinical practice particularly in low- or middle-income countries.

Allogeneic stem cell transplantation (allo-HSCT) remains the only curative treatment for Myelodisplastic Syndromes (MDS) and many factors may predict outcomes as cell source, conditioning regimen and donor type. Haploidentical HSCT is an alternative in the absence of an HLA-matched donors. In generall, clinical studies comparing recipient outcomes after allo-SCT with HID versus MSD or MUD in myeloid malignancies have suggested similar outcomes, with an overall survival (OS) between 40% and 80%, but few studies focused on the outcomes after allo-HSCT for MDS patients (excluding AML).

ABSTRACTAegla castro, is an endemic anomuran crab from Brazil found in headwater streams of the Atlantic Forest on the Paranapanema River basin. This study aims to evaluate the gonadal maturity of A. castrobased on macroscopic and microscopic analyses. Crabs were collected in Mauá da Serra (23º57’ S; 51º06ʹ W), Paraná, Brazil in the winter and spring of 2015, and summer, autumn, and spring of 2016. Individuals had their gonads analysed and categorized into stages of development. Stage1, ovaries are macroscopically undetectable through abdominal transparency, while ovaries of stages 2 and 3 could be detected through abdominal transparency and have characteristic colour and length in relation to the abdominal somites. Ovaries have pre-vitellogenic and vitellogenic oocytes in all stages of development, with germ cells and structural epithelium characteristic of these gonadal maturation phases. Spermatogenesis in males begins in spring, with the production of spermatozoa in autumn and spermiation in winter. Aegla castrohas seasonal reproduction. The macroscopic stages of gonadal development correspond to the histological gonadal maturation, indicating that the macroscopic observation of the gonads of live animals is feasible in studies of the reproductive cycle of aeglids.

ABSTRACTRoot-knot nematode Meloidogyne mayaguensisRammah & Hirschmann is a limiting factor in guava tree (Psidium guajavaL.) production around the world. An alternative to address this problem is the use of resistant root-knot nematode rootstocks. However, the guava nursery system which is based on herbaceous stem cutting propagation needs to be modified as a rootstock produced via cutting must be grafted with nematode susceptible commercial cultivars. Thus, the objective of this study was to verify the feasibility of omega bench grafting in guava tree propagated by herbaceous stem cutting. Root-knot nematode tolerant ‘Tailandesa’ herbaceous stem cuttings was used as rootstock and ‘Paluma’ and ‘Pedro Sato’ as commercial cultivar grafts. It was observed 100% rooting, cutting survival, and leaf retention on rootstocks grafted with ‘Paluma’ and ‘Pedro Sato’. However, grafting take was not successful as the tissues presented fast oxidation, dehydration and graft death in both commercial cultivars. However, the immersion of the grafts into a solution containing 2.0% citric acid and 1.0% ascorbic acid controlled the oxidation process and resulted in 28.6% and 37.5% successful grafting in the combinations ‘Paluma’/‘Tailandesa’ and ‘Pedro Sato’/’Tailandesa’, respectively.

The morphologies of only 10 of the 93 described species of AeglaLeach, 1820 are known. We describe and illustrate the external morphology of newly-hatched, first-stage juveniles of Aegla jacutingaMarçal & Teixeira in Marçal, Páez, Souza-Shibatta, Sofia & Teixeira, 2020 using light microscopy and scanning electron microscopy (SEM) comparing them with the same stage in other congeners and providing diagnostic characters. Aeglais morphologically a conservative genus, which creates many problems in the identification of its many cryptic species. It thus becomes necessary to refine the taxonomic characters used in the taxonomy of the group by providing a new sets of morphological characters to be analyzed.

Weld metal transfer modes in gas metal arc welding process have an important role in electric arc stability, in the amount of absorbed gases by the melted metal in weld pool, allow out-of-position welding and influence in sparkles generation. The objective of the present work is to obtain images of the metal transfer to the weld pool, in order to better characterize the transfer mode boundaries. Besides, electric signals of welding current and voltage were acquired and compared with the generated images to confirm the results. High-speed imaging helps to identify the weld metal transfer mode. In this work, a high-speed camera and filter lenses were used to get images and movies of welding arcs generated by three different AWS ER70S-6 wire diameters (0.8, 1.0 and 1.2 mm). Also, different shielding gases and mixtures were tested (Ar, Ar + 2% O2, Ar + 10% CO2, Ar + 25% CO2and CO2). Metal transfer modes could be observed at a frame rate of 5000 fps. Metal transfer mode maps were plotted to identify regions of each kind of metal transfer. Interchangeable globular/spray metal transfer occured in some cases of cyclic alternation between globular and spray transfer during the welding.

Consumers around the world appreciate strawberries for their taste. They have low calories and high concentrations of soluble fibers, vitamin C and flavonoids. This paper verified the combined effect of O2, CO2, and N2O levels on 'Oso Grande' strawberries stored at 10° C, under controlled atmosphere (CA). Five different gas mixtures were used: 0.03 kPa CO2 + 20 kPa O2, 80 kPa N2O + 20 kPa O2, 90 kPa O2 + 10 kPa N2, 60 kPa O2 + 40 kPa CO2, and 20 kPa O2 + 20 kPa CO2 + 60 kPa N2O. The lowest incidence of postharvest decay was observed with treatment 20 kPa O2 + 20 kPa CO2 + 60 kPa N2O, followed by 80 kPa N2O + 20 kPa O2 and 90 kPa O2 + 10 kPa N2. The treatment 60 kPa O2 + 40 kPa CO2 induced an increase in the production of acetaldehyde and ethanol, and these levels were considered inadequate for human consumption. The first factor, named senescence, displayed a positive correlation with soluble solids, luminosity, hue angle, firmness and incidence of decay. The second factor, named CA-induced injury, showed that total acidity correlated negatively with ethanol and acetaldehyde levels. Hierarchical cluster analysis indicated that strawberries stored under 80 kPa N2O + 20 kPa O2 for 14 days more closely resembled the quality of fresh fruit at the moment of harvest. 'Oso Grande' strawberries stored at 10 °C under 80 kPa N2O + 20 kPa O2 and 90 kPa O2 + 10 kPa N2 were in better conditions, with no metabolic alterations, showing that these are the ideal storage conditions. [ABSTRACT FROM AUTHOR]

The main definition for meat quality should include factors that affect consumer appreciation of the product. Physical laboratory analyses are necessary to identify factors that affect meat quality and specific equipment is used for this purpose, which is expensive and destructive, and the analyses are usually time consuming. An alternative method to performing several beef analyses is near-infrared reflectance spectroscopy (NIRS), which permits to reduce costs and to obtain faster, simpler, and nondestructive measurements. The objective of this study was to evaluate the feasibility of NIRS to predict shear force [Warner-Bratzler shear force (WBSF)], marbling, and color (*a = redness; b* = yellowness; and L* = lightness) in meat samples of uncastrated male Nelore cattle, that were approximately 2-yr-old. Samples of longissimus thoracis(n= 644) were collected and spectra were obtained prior to meat quality analysis. Multivariate calibration was performed by partial least squares regression. Several preprocessing techniques were evaluated alone and in combination: raw data, reduction of spectral range, multiplicative scatter correction, and 1st derivative. Accuracies of the calibration models were evaluated using the root mean square error of calibration (RMSEC), root mean square error of prediction (RMSEP), coefficient of determination in the calibration (R2C), and prediction (R2P) groups. Among the different preprocessing techniques, the reduction of spectral range provided the best prediction accuracy for all traits. The NIRS showed a better performance to predict WBSF (RMSEP = 1.42 kg, R2P = 0.40) and b* color (RMSEP = 1.21, R2P = 0.44), while its ability to accurately predict L* (RMSEP = 1.98, R2P = 0.16) and a* (RMSEP = 1.42, R2P = 0.17) was limited. NIRS was unsuitable to predict subjective meat quality traits such as marbling in Nelore cattle.

AbstractSpecimens of Aegla castroSchmitt, 1942 were monthly sampled from June 2012 to May 2013 from Couro stream (23°57′15″S 51°06′00″W), located in the Upper Paraná river region, southern Brazil. Population structure, morphometric and functional maturity were analyzed. Allometric growth analysis on chela dimension versus carapace length (CL) was employed to recognize juveniles and adult individuals. Two sequential groups of adult males (morphotypes I and II) were recognized according to the state of development of the pair of claws. The CL where 50% of the population of individuals are adults (CL50) was used to estimate the size at the onset of morphometric maturity. Males attain morphometric maturity at same size class of females (8.0–9.0 mm CL). Males and females are heterochelous and they often show the left chela more developed. The reproductive period was extended from June to September 2012 and April to May 2013 (six months) with record of 22 ovigerous females. The recruitment occurred in the months following the reproductive period, with higher records in October and December 2012. All the information gathered here should be useful to better understand the biology of the species studied.

This study proposes a rapid and non-destructive method of jaboticaba [Myrciaria cauliflora(Mart.) O. Berg] fruit classification at three maturity stages based on the skin colour (immature – fruit completely green, physiologically mature – fruit turning from green to purple, and ripe – fruit completely purple) using Near-Infrared Reflectance Spectroscopy (NIRS) combined with principal component analysis-linear discriminant analysis (PCA-LDA), and variable selection techniques employing a successive projection algorithm (SPA-LDA) or genetic algorithm (GA-LDA). One hundred eighty jaboticaba fruit samples in three maturity stages were used and the multivariate classification accuracy results were tested based on sensitivity, specificity, positive (or precision) and negative predictive values, Youden index, and positive and negative likelihood ratios. In the immature stage the classification models PCA-LDA, GA-LDA and SPA-LDA achieved a sensitivity of 100% in the validation set. The results obtained in this study suggest that the proposed method is a promising alternative for assessing the jaboticaba fruit maturity, opening the possibility for automation in packing houses.

Aguardente is a typical Brazilian spirit produced by the distillation of sugarcane. The valorisation of this spirit can be attributed to its notoriety, related to its production origin which can influence its quality. Therefore, the objective of this study was to use NIR spectroscopy coupled with discriminant analysis as a non-destructive method to validate the authenticity of aguardentes produced in two geographic regions and to predict their ethanol content. Some chemometric methods were used to discriminate the sugarcane aguardente, namely partial least squares-linear discriminant analysis (PLS-LDA), principal component analysis-linear discriminant analysis (PCA-LDA), and variable selection techniques such as successive projection algorithm (SPA-LDA) and genetic algorithm (GA-LDA). NIR spectra were collected using a FT-NIR spectrometer (4000–10 000 cm−1) with a spectral resolution of 16 cm−1, 8 cm−1intervals, and 64 scans. The PCA results were not effective in classifying the aguardente samples, but with PLS-DA, PCA-LDA, SPA-LDA, GA-LDA and LDA it was possible to obtain an 87.2% prediction accuracy. Better results were obtained using PLS-DA on raw spectra and GA-LDA using only six wavelengths (namely 1025 nm, 1181 nm, 1596 nm, 1610 nm, 1653 nm, 2125 nm) which gave a relatively good accuracy rate (up to 87.2%). NIR spectroscopy and chemometrics can be used as a non-destructive method to validate authenticity, and PLSR combined with NIR was a good non-destructive method to predict ethanol content in sugarcane aguardente.

The reproductive capacity of the swimming crab Callinectes danaeSmith, 1869 was assessed based on the reproductive investment (RI) of ovigerous females and the gonadosomatic index (GI) of non-ovigerous adult females. Crabs were collected in June 2009 with a shrimp fishing boat at Ubatuba (23° 26′S 45° 02′W) São Paulo, Brazil. Overall, 191 adult females were analyzed, of which 108 were ovigerous and 83 non-ovigerous. The size of ovigerous females ranged from 54.5 to 79.8 mm carapace width (CW) and non-ovigerous females ranged from 56.0 to 80.9 mm CW. RI values did not differ among the size classes (ANOVA, p > 0.05), with an overall mean of 15.8%. The same occurred with the Gonadossomatic index for non-ovigerous females, for which the mean value was 11.7%. The regression between the dry weight of the egg mass and the dry weight of the crab (Student’s t, p = 0.25; ttab = 1.66; tcal = 0.66) indicated an isometric relationship for the variables analyzed. The simultaneous occurrence of gonadal maturation and embryonic development in ovigerous females suggests that these crabs may spawn at least twice in the same breeding season and reveals the need for evaluation of the allocation of energy in subsequent spawns.

The distribution and abundance of marine organisms are directly or indirectly a function of biotic and abiotic features such as sediment and water properties. In this study we analyzed the spatial and temporal distribution of Callinectes danaein two distinct littoral areas of the northern coast of the State of São Paulo, Brazil and associated them with local geomorphologic features and environmental factors. We collected specimens on a monthly basis, from July 2001 to June 2003, using a fishing boat equipped with double-rig net, in two locations: Ubatuba (UBA) and Caraguatatuba (CAR). Collecting was carried out in seven transects with different depths, which were further subdivided into two regions: bay region (5, 10, 15 and 20 m deep); and inner shelf region (25, 30 and 35 m deep). Samples from different periods and transects differed significantly in abundance. A positive correlation was found between the abundance of C. danaeand the following parameters: temperature at the bottom, organic matter content and Phi (grain diameter). By contrast, a negative correlation was established between specimen abundance and salinity at the bottom. The total abundance was highest in 5 m deep transects in both regions. Summer was the season when individuals were least abundant in both areas. We concluded that the spatial and temporal distribution of C. danaeare determined by a set of environmental factors and not just by one single factor.

Este artículo tiene como objetivo buscar una breve reflexión sobre la relación entre la protección internacional del medio ambiente y los derechos humanos y analizar los aportes del llamado greeningdel sistema interamericano de derechos humanos, un fenómeno que se produce cuando derechos ambientales son protegidos a través de la Convención Americana sobre Derechos Humanos, que en principio se suponen para garantizar los derechos civiles y políticos.

The population biology of Nematopalaemon schmitti in the Ubatuba region on the northern coast of the state of Sao Paulo was characterized through analyses of the length-frequency distribution, sex ratio, reproductive period, and juvenile recruitment. The specimens were caught monthly from January 1998 to December 1999, from a shrimp boat equipped with double-rig trawl nets. A total of 1073 specimens were analyzed, and the sex and carapace length (0.1 mm) of each individual were noted. The analyzed specimens consisted of 152 juveniles, 437 adult males, 296 adult females, and 188 ovigerous females (OFs). The monthly distribution of N. schmitti by size classes revealed a stable population structure, with both juveniles and adults continuously present. This population showed a unimodal distribution; however, bimodality was identified in June 1998 and 1999 for males, and June 1998 and July 1998 and 1999 for females, probably related to recruitment pulses of juveniles. Sexual dimorphism was indicated by the presence of females in the larger size classes, probably because of their growth rate. The proportion between males and females differed from 1 : 1 in certain size classes and seasons of the year (χ2, p < 0.05); in some situations, the females were more abundant than the males, or vice versa. No significant relationship was detected between the seasonal mean temperatures of the bottom water and the percentages of OFs and young, or for the relationship between these two groups (Spearman, p > 0.05). The continuous presence of OFs and young in the population suggests a pattern of continuous reproduction for N. schmitti in the Ubatuba region.

The reproductive biology of Persephona mediterranea was investigated in relation to the following aspects: population structure, size at the onset of sexual maturity, sexual size dimorphism, and reproductive period. A total of 1,283 specimens were obtained (565 males, 120 non-ovigerous females and 598 ovigerous females). CW50 values were 22.8 and 22.3 mm CW for males and females, respectively, and the mean size of males was significantly larger than that of females. Persephona mediterranea shows continuous reproduction. However, there is a reduction in the proportion of ovigerous females and males with gonads in advanced developmental stage during the autumn and beginning of the winter. There was no significant correlation between the reproductive activity and variation in abiotic factors. Of the total captured specimens, 98.99% were sexually mature, which may indicate a differential spatial distribution among the distinct age groups, and sampling sites are probably areas occupied by adult individuals of this population.

Chromophore identification in biological samples often requires the physical separation of the compounds, which can be difficult. Although there are several advantages to hyphenated spectroscopic techniques for identification of substances, complex mixtures of chromophores presenting overlapped spectra cannot be identified directly through this method. This work presents an application of chemometrics to compound identification in biological samples by a spectroscopic hyphenated technique using a curve resolution method. The PARAllel FACtor analysis model (PARAFAC), which has no rotational indeterminacy, was used for curve resolution of excitation-emission spectra of human dental tartars. PARAFAC was applied under constraints (i.e., unimodality and non-negativity) and evaluated with a validation procedure. The resolved profiles are porphyrinic-like spectra presenting excitation band maxima at 407, 416, and 431 nm in the Soret band region (390–440 nm) of these substances.

We characterized the natural diet and feeding dynamics of Aegla castroSchmitt, 1942(Aeglidae), an endemic freshwater crab. Research was undertaken on the trophic spectrum of the species by analyzing the stomach contents of individuals collected in a stream in southeastern Brazil, and evaluating the diet through the index of relative importance (IRI). Seasonal and circadian variations in diet composition were analyzed, and the feeding dynamics investigated through repletion index (RI) analysis. We analyzed a total of 240 stomachs and identified 20 main food categories of the diet, which were studied throughout the seasons. Aegla castromaintained a constant RI throughout the year, with a peak of food activity between midnight (00) and 0600. The species was characterized as an omnivorous generalist and opportunist. The high overlay in the diet composition of adults of both sexes and juveniles indicates that they share the same food resources, even though they seem to exploit them in different quantities and frequencies. Future research should focus on the species of Aeglawhose diets have not yet been described, and use available data to inform trophic network analyses and models.

Bacuri fruit at two stages of maturation, according to skin color (green and yellow), harvested in Teresina-PI, Brazil, were characterized for the following: weight of whole fruit, skin, pulp and seeds; fruit diameter, length and volume. The pulp was analyzed for moisture content; soluble solids (SS), soluble sugars; reducing sugars, titratable acidity; PH; starch; pectins; vitamin C; phenolics; minerals (P, K, Ca, Mg, Fe, Cu, Mn and Zn) and the activity of polygalacturonase (PG). Pulp yield varied from 16.29% to 18.35%. SS content was higher (16,8°Brix) in yellow fruit than in green fruit (p<0,01). Reducing sugars represented approximately 30% of total soluble sugars. Yellow fruit contained more cell wall material (CWM) and higher PG activity in the pulp, while green fruit contained more protein, pectin in CWM, K and P. No differences were found as related to the other characteristics between green and yellow fruit, suggesting that both can be used for fresh consumption. Juice processing may be impaired by the starch content in bacuri. [ABSTRACT FROM AUTHOR]

Sickle Cell Diseases (SCD) are the most frequent inherited monogenic diseases worldwide. Data from the neonatal screening program showed that about 4% of the brazilian population harbors the sickle cell trait, reaching until 10% in the afro-brazilians. In the northern states of Brazil this frequency is much higher, as an example in Bahia 1/17 births are of children with sickle cell trait. The neonatal screening program doesn't cover the whole country so the estimate of about 3500 newborns with SCD per year in Brazil is certainly underestimated. Nevertheless the first official document regarding the treatment of these patients was published by the brazilian ministry of health only in 2004. Hydroxiurea (HU) is already used in more severe forms of SCD by the public health system since several years but is not available for all patients yet. In a recent publication Machado et al (ASH 2010 #843) demonstrated for the first time that HU can prolong survival in SCD, although the mortality rate for this disease is still high in Brazil. Stem Cell Transplanation (SCT) is the only available curative treatment for the inherited hemoglobinopathies, in spite of this there is no such program for SCD in Brazil. Here we describe the first brazilian cases transplanted in 5 brazilian centers of severely affected patients with no other treatment options not responding to HU.Sixtheen patients (15 HbSS, 1 case Sbeta thalassemia), median age 16 years (3–39), all except for one with HLA identical sibling donors (3 donors with sickle cell trait)received a alo SCT. Indications consisted mainly of severe acute chest syndrome, priapism, alloimunization and silent cerebral infarctions and overt previous stroke. One patient transplanted because of advanced Hodgkin's disease. The conditioning consisted of BuCy or FluBu with ATG in all but 3 patients who received FluCy and ATG. The decision was based on the age of one of the patients (39 years old and grade IV liver fibrosis) and 2 patients with previous stroke.Primary graft failure occurred only with the unrelated donor. All other patients engrafted, but one patient conditioned with FluCy lost the graft about 100 days after transplant. She received a second transplant 3 years after with BuCy from the same donor, had a 100% engraftment without any sign of GVHD but unfortunately died 3 years later in remission from a SNC bleeding caused by her acquired vascular abnormality. Median follow up for all surviving patients is 2 years (152 days – 12,2 years). Overall survival is 13/16. Death occurred in 3 cases (1 primary graft failure and sepsis, 1 Hodgkin disease, 1 hemorrhagic stroke in a patient with severe cerebral vasculopathy with MoyaMoya). No veno-occlusive disease was observed. Acute GVHD grade II were observed in 4 patients (gut and skin) easily treated with short time prednisone. All surviving patients but one are full chimeras and the 39 years old patient remain a stable mixed chimera 6 years after transplant. Quality of life has improved in all patients considerably referring extremely grateful to be free of pain.Different from thalassemia SCT can be offered safely for older patients with SCD in our opinion. Our data confirm the curative potential of SCT in SCD and reinforce us to offer this curative approach to patients not responding to HU. A prospective trial is planned in Brazil who should address also long term toxicities and quality of life.No relevant conflicts of interest to declare.

Cyclophosphamide (Cy) with/without antithymocyte globulin (ATG) as conditioning regimen for allogeneic hematopoietic stem cell transplantation (AlloHSCT) is the treatment of choice for young patients with severe aplastic anemia (SAA). In developing countries, and particularly in Brazil, ATG costs limit its use in AlloHSCT for SAA patients. Alternative low-cost regiments, like busulfan (BU) with Cy as conditioning regimen is still associated with a significant rate of rejection, especially in heavily transfused patients, and long-term infertility. Alemtuzumab (Cam) was reported as an alternative to ATG for SAA patients, with similar activity and a lower cost.In order to study the effect of the combination of Cy and Cam, we reviewed all AlloHSCT performed for SAA using this conditioning regimen. Between April 2007 and Mai 2009, fifteen patients with SAA (defined by Camitta criteria) underwent an AlloHSCT in our institution. Median age at transplantation was 25 (range 5-42) years. All but one patient had positive CMV serology. Median number of transfusions was 20 (range 10-67). One patient received a second AlloHSCT due to a late (> 4 years) graft rejection. Patients received an unmanipulated bone marrow (n=11) or peripheral blood (n=4) graft as stem cell source and all but one patient were transplanted with an HLA-identical sibling. Median number of nucleated cell infused was 2.86 (range 1.65-6.50)x10 8/kg. Cyclosporin alone (n=10) or in combination with methotrexate (n=5) was used as GVHD prophylaxis.Thirteen out of 15 patients presented neutrophil recovery with a median time to > 0.5×10 9 neutrophil/L of 23 (range 13-30) days. Platelet recovery (> 20×10 9 platelets/L) occurred in thirteen patients with a median time of 16.5 (range 9-45) days. Acute graft versus host disease (GVHD) was observed in just one patient (grade II). None of 12 patients alive 100-days after AlloHSCT presented chronic GVHD. Seven patients presented CMV reactivation. One patient did not engrafted and other presented a late (14 months) rejection. One patient became pregnant after alloHSCT and gave birth to a healthy child. With a median follow-up of 315(range 4-782) days, two patients died and the estimate 1-year overall survival rate is 87%. One patient died due to complications of a CNS bleeding that occurred hours before marrow infusion and the other of GI infection while still on neutropenia.Use of cyclophosphamide and alemtuzumab as conditioning regimen is a valid option in SAA patients undertaking AlloHSCT, with significant lower rates of acute and chronic GVHD. Nevertheless, a longer follow-up is required to properly evaluate rejection incidence.Off Label Use: Drug: Alemtuzumab Off-label Use: Aplastic Anemia.