Generation, establishment and characterization of a pluripotent stem cell line (CVTTHi001-A) from primary fibroblasts isolated from a patient with activated PI3 kinase delta syndrome (APDS2).

APDS2 is caused by mutations in PIK3R1 gene resulting in constitutive PI3Kδ activation. PI3Kδ is predominantly expressed in leukocytes and plays critical roles in regulating immune responses. Here we first derived fibroblast primary cells from a skin biopsy of a patient carrying a heterozygous single T deletion in intron 11 of the PIK3R1 gene. We next present the derivation of an induced pluripotent stem cell (iPS) line using a non-integrative reprogramming technology. Pluripotent-related hallmarks are further shown, including: iPSCs self-renewal and expression of pluripotent and differentiation markers after in vitro differentiation towards embryonic germ layers, assessed by RT-PCR and immunofluorescence.

Adult peripheral blood and umbilical cord blood NK cells are good sources for effective CAR therapy against CD19 positive leukemic cells.

Among hematological cancers, Acute Lymphoblastic Leukemia (ALL) and Chronic Lymphocytic Leukemia (CLL) are the most common leukemia in children and elderly people respectively. Some patients do not respond to chemotherapy treatments and it is necessary to complement it with immunotherapy-based treatments such as chimeric antigen receptor (CAR) therapy, which is one of the newest and more effective treatments against these cancers and B-cell lymphoma. Although complete remission results are promising, CAR T cell therapy presents still some risks for the patients, including cytokine release syndrome (CRS) and neurotoxicity. We proposed a different immune cell source for CAR therapy that might prevent these side effects while efficiently targeting malignant cells. NK cells from different sources are a promising vehicle for CAR therapy, as they do not cause graft versus host disease (GvHD) in allogenic therapies and they are prompt to attack cancer cells without prior sensitization. We studied the efficacy of NK cells from adult peripheral blood (AB) and umbilical cord blood (CB) against different target cells in order to determine the best source for CAR therapy. AB CAR-NK cells are slightly better at killing CD19 presenting target cells and CB NK cells are easier to stimulate and they have more stable number from donor to donor. We conclude that CAR-NK cells from both sources have their advantages to be an alternative and safer candidate for CAR therapy.

Optimization of the management of platelet concentrate stocks in the Basque Country using mathematical simulation.

BACKGROUND AND OBJECTIVES: The management of platelet concentrate (PC) stocks is not simple given their short shelf life and variable demand. In general, managers decide on PC production based on personal experience. The objective of this study was to provide a tool to help decide how many PC units to produce each day in a more rational and objective way. MATERIALS AND METHODS: From the historical data on PCs produced, transfused and discarded in the Basque Country in 2012, a mathematical model was built, based on the normality of the time series of the transfusions performed on each day of the week throughout the year. This model was implemented in an easy-to-use Excel spreadsheet and validated using real production data from 2013. RESULTS: Comparing with real 2013 data, in the best scenario, the number of PC units that expired was 87·7% lower, PC production, 14·3% lower and the age of the PCs transfused nearly 1-day younger in the simulation. If we want to ensure a minimum stock at the end of each day, the outdating rate and average age of the transfused PCs progressively increase. CONCLUSION: The practical application of the designed tool can facilitate decision-making about how many PC units to produce each day, resulting in very significant reductions in PC production and wastage and corresponding cost savings, together with an almost 1 day decrease in the mean age of PCs transfused.

Characterization of a novel HLA-DRB1 allele: DRB1*11:153.

HLA-DRB1*11:153 differs from DRB1*11:131 by one nucleotide at position 286 where C > A, (codon 67 CTC>ATC), resulting in an amino acid substitution, 67L>67I.

Sequencing of two novel HLA-A*29 alleles, A*29:39 and A*29:40.

A*29:39 differs from A*29:02:01 by three clustered amino acid replacements at α1 domain, T73>I73, A76>V76 and N77>D77. A*29:40 shows one nucleotide difference regarding A*29:02:01 allele, resulting in one amino acid substitution at position 154, E154>G154.

Translation and psychometric testing of the Basque version of the SF-36 health survey.

INTRODUCTION: The primary goals of this study were to adapt the Short Form-36 (SF-36) questionnaire to the Basque language and to assess the translated questionnaire's psychometric properties. METHODS: Two pilot studies and a field study were conducted in 1999. The pathologies selected were hypertension, eating disorders, back pain, and HIV infection; blood donors were also included. The analyses conducted at item-level were: item internal consistency, item discriminant validity, equal item variance, and equal item-scale correlation. Internal consistency was measured at the scale level. Reproducibility, convergent validity and discriminant validity were also examined. RESULTS: 285 individuals took part in the study; sufficient data were obtained for 265 (93%). All items correlated higher than 0.4 with their hypothesized scales. Within each scale, item standard deviations were similar. Item-scale correlations were also similar. Cronbach's alpha coefficients varied from 0.76 to 0.92. In the test-retest reliability study, which included 54 additional individuals, all scales were above 0.50. Regarding the inter-scale correlation, the majority exceeded the 0.40 coefficient. In the correlation of similar scales of the SF-36 and the Nottingham Health Profile, intraclass correlation coefficient results ranged from 0.29 to 0.62. CONCLUSIONS: These results confirm that this new version of the SF-36 has been translated and adapted correctly for the Basque language and that it fulfils, at least partially, the psychometric properties required for this instrument. Further studies, however, are needed to completely validate the Basque version of the SF-36.

[The impact of the implementation of staying in the United Kingdom for six months or more as donor exclusion criteria on the donor base of the Basque Country]. / Repercusión de la implantación de la estancia en el Reino Unido durante seis o más meses como motivo de exclusión sobre la base de donantes de sangre en el País Vasco.

BACKGROUND: To reduce the risk of new variant Creutzfeldt-Jakob disease by blood products some countries exclude persons who have spent six months or more cumulatively in the United Kingdom as blood donors. METHOD: We asked our donors about this selection criteria to evaluate the loss of blood donors and donations in case of implementation of this measure. RESULTS: 11,681 donors and 1,648 refused persons were analyzed. 123 (1.05%) and 11 (0.66%) respectively fulfilled this criteria. CONCLUSIONS: In case of implementation of this selection criteria 1% of blood donors and 1.77% blood donations would be lost.

[Report on the activity of the blood bank accreditation program (1987-1995)]. / Balance de la actividad del programa de acreditación de Bancos de Sangre (1987-1995).

PURPOSE: To show the incidence of the deficiencies detected in the Blood Banks for the accreditation by the Transfusion Accreditation Committee (CAT), previously named PABAS. MATERIALS AND METHODS: Analysis of the reports of the accreditation of 85 Blood Banks made by the PABAS during the period 1987-1995. RESULTS: Eighty-five (20.8%) of the 407 Community Blood Centers, Hospital-Based Blood Banks and Transfusional Services of Spain had been surveyed. There were 244 deficiencies, of which 31 (12.7%) were of the equipment, 114 (46.7%) of the procedures used, and 99 (40.6%) of the documentation. The activities with more incidence of faults were: Control of the temperatures of the storage of units 53 (21.7%), label of the components 38 (15.5%), quality system of the institution surveyed 32 (13.1%), transfusional procedures 30 (12.3%), and on the procedure of the selection of donors 29 (11.9%). By contrary, the areas of work with fewer incidences of faults were those related with the collection of the blood and components 10 (4.1%) and the laboratory 14 (5.7%). CONCLUSIONS: Low percentage of the Community Blood Centers, Hospital-Based Blood Banks and Transfusional Services, which ask to be accredited by the Transfusion Accreditation Committee. The 83.7% of the errors detected are of the procedures and documentation, which could be easily corrected by the training and continuous improving of the quality, and without need of new inversions in equipment.