Se determinó la actividad de las vías clásica y alternativa del complemento, así como la cuantificación de algunos de sus componentes en 46 pacientes con anemia drepanocítica (AD) (hemoglobina SS), ...21 sin complicaciones clínicas y 25 con estas; entre las más frecuentes encontramos: número de infecciones, úlceras maleolares, crisis vasooclusivas, hepáticas y aplásticas, considerando como límite un año anterior a la toma de muestra de sangre. Se demostró una disminución significativa de la actividad de la vía alternativa, factor B y del componente C3 en el grupo de pacientes con complicaciones clínicas. Se observó una correlación significativa entre el número de crisis vasooclusivas e infecciones y la actividad de la vía alternativa, el factor B y el C3. Estos resultados sugieren que los fenómenos inflamatorios que pueden persistir en pacientes con AD en estado basal, fundamentalmente aquellos con historia anterior de complicaciones clínicas, pueden provocar alteraciones en la actividad del complementoThe activity of the classic and alternative complement pathways, as well as the quantitation of some of their components was determined in 46 patients with sickle cell anemia (haemoglobin SS), 21 with no clinical complications and 25 with them. Among the most frequent complications we found: number of infections, malleolar ulcers, and vaso-occlusive, hepatic and aplastic crises. A year previous to the blood sample taking was considered as the limit. A significant reduction of the activity of the alternative pathway, factor B and component C3 was proved in the group of patients with clinical complications. A marked correlation was observed between the number of vaso-occlusive crises and infections and the activity of the alternative pathway, factor B and complement C3. These results suggest that the inflammatory phenomena that may persist among patients with basal sickle cell anemia, mainly those with prior history of clinical complications, may produce alterations in the complement activity
Se efectuó la determinación de inmunocomplejos circulantes (ICC) así como la medición del sistema complemento por la vía clásica, vía alterna, actividad del factor B y la cuantificación del tercer ...(C3) y cuarto (C4) componentes de complemento en 30 pacientes con leucemia promielocítica (LPM) al diagnóstico, 22 de los cuales presentaron coagulación intravascular diseminada (CID). Se demostró la existencia de niveles elevados de ICC en los enfermos con CID y una disminución significativa de la actividad de la vía clásica, los componentes C3 y C4 en los enfermos con CID, al compararlos con el grupo de pacientes que no presentaba el trastorno de la hemostasia y los controles normales, lo que sugiere la posible participación de estos parámetros en el fenómeno de la CID en estos enfermos
Se realizó un estudio longitudinal para detectar anticuerpos anticitoplasma de neutrófilos (ANCA) en 13 pacientes con anemia drepanocítica en crisis vasooclusiva y en estado basal, mediante un método ...de inmunofluorescencia indirecta. Del total de 34 muestras de suero obtenidas, 16 fueron en crisis vasooclusiva y en 12 de ellas, correspondientes a 10 pacientes, se demostró la presencia de p-ANCA. En el resto de las muestras en crisis vasooclusiva y en estado basal no se observó la presencia de p-ANCA o c-ANCA. Los resultados obtenidos sugieren la posible participación de los p-ANCA en el daño isquémico, así como la importancia de su medición en el diagnóstico de las crisis vasooclusivas (CVO) en los pacientes con anemia drepanocítica (AD)A longitudinal study was made to detect antineutrophil cytoplasmic antibodies (ANCA) in 13 patients with sickle cell anemia in vasocclusive crisis and basal state by using an indirect immunofluorescence method. Of 34 serum samples, 16 were in vasocclusive crisis and 12 of them corresponding to 10 patients revealed the presence of p-ANCA. Neither p-ANCA nor c-ANCA was observed in the rest of the samples taken in vasoclussive crisis and in basal state. The results achieved signaled a possible involvement of p-ANCA in ischemic damage as well as the importance of their measurement in the diagnosis of vasocclusive crisis in patients with sickle cell anemia
Objetivo: el objetivo de esta evaluación es identificar si hay asociación entre las deleciones del gen IKZF1, características clínicas y desenlaces en menores de 18 años con leucemia linfoblástica ...aguda. Materiales y métodos: se incluyeron 130 pacientes menores de 18 años con diagnóstico confirmado de leucemia linfoblástica aguda que consultaron de manera consecutiva en un centro de referencia entre junio de 2018 y agosto de 2020. La identificación de deleciones de IKZF1 se realizó utilizando la prueba MLPA (multiple ligation-dependant amplificaction) SALSA MLPA P335 ALL_IKZF1 probemix.
Se efectuó la determinación de inmunocomplejos circulantes (ICC) así como la medición del sistema complemento por la vía clásica, vía alterna, actividad del factor B y la cuantificación del tercer ...(C3) y cuarto (C4) componentes de complemento en 30 pacientes con leucemia promielocítica (LPM) al diagnóstico, 22 de los cuales presentaron coagulación intravascular diseminada (CID). Se demostró la existencia de niveles elevados de ICC en los enfermos con CID y una disminución significativa de la actividad de la vía clásica, los componentes C3 y C4 en los enfermos con CID, al compararlos con el grupo de pacientes que no presentaba el trastorno de la hemostasia y los controles normales, lo que sugiere la posible participación de estos parámetros en el fenómeno de la CID en estos enfermosThe determination of the circulating immunecomplexes (CIC) as well as the measurement of the complement system were carried out by the classical pathway, alternate pathway, factor B activity and the quantitation of the third (C3) and fourth (C4) components of the complement in 30 patients with promyelocytic leukaemia (PML) on diagnosis, 22 of whom presented disseminated intravascular coagulation (DIC). It was proved the existance of elevated levels of CIC in patients with DIC and a marked reduction of the ativity of the classical pathway and of the C3 and C4 components in patients with DIC, on comparing them with the group of patients that did not have hemostasis disorder and with the normal controls, which suggest the possible participation of these parameters in the phenomenon of DIC in these patients
Lipid abnormalities may contribute to chronic allograft nephropathy (CAN). Apolipoprotein E (ApoE) gene polymorphism regulates lipoprotein metabolism, but little is known about an association between ...CAN and this polymorphism. The ApoE gene (E3/E4) polymorphism was typed by PCR assay (99 E3/E3, 28 E3/E4, 1 E4/E4) on 128 consecutive renal transplant patients with functioning grafts for more than 3 years (6.7 ± 2.8 years). Twenty-eight patients with histological CAN were compared with 100 patients who had no clinical evidence of chronic rejection (no proteinuria and sCr < 2.5 mg%). As expected, univariate analysis revealed that patients with CAN experienced a greater acute rejection rate (78% vs 21%;
P = .001), a higher serum creatinine (3.6 ± 1.7 vs 1.4 ± 0.5 mg%;
P = .0001), and an older organ donor (43 ± 20 vs 29 ± 13 years;
P = .0001). The lipid profiles (total cholesterol and triglycerides levels) were similar in both groups with 60% in each group receiving anti-lipemic drugs. Interestingly, the ApoE epsilon 4 allele was overrepresented in the group with CAN (39% vs 17%,
P = .019). Logistic regression analysis showed that the epsilon 4 allele was an independent predictor of CAN (OR: 3.4; CI 95%: 1.07 to 11;
P = .040) as were donor age and acute rejection episodes. In conclusion, an interaction between risk factors and genetic factors may determine CAN in this population. This finding may help to target prophylactic interventions in these recipients.
Glycoprotein IIIa/IIb is a membrane receptor for fibrinogen and von Willebrand factor that plays an important role in platelet aggregation. The beta integrin chain of this receptor, GPIIIa, is ...polymorphic, and the allele known as PlA2 has been associated with coronary thrombosis. The GPIIIa genotype of a cohort of 119 consecutive renal allograft recipients (46.3 +/- 13 yr; 85 M/34 F; 24.4% diabetic patients) was determined by PCR-restriction fragment length polymorphism, and those patients were followed for at least 12 mo. From 119 patients with at least 1 yr of follow-up, those who suffered an acute rejection (n = 52) showed a lower proportion of HLA-DR beta1 identity with the donor (7.7% versus 23.9%; P = 0.03), a higher proportion of cytomegalovirus-positive (CMV+) donors/CMV- recipients (21% versus 7.5%; P = 0.05), and the PlA2 allele was more frequent (48.1% versus 26.9%; P = 0.02) compared with patients free of acute rejection (n = 67). No other variable was associated with acute rejection in the univariate analysis. The impact of the three above-mentioned significant variables on acute rejection was analyzed by stepwise logistic regression. The presence of the PlA2 allele yielded an odds ratio of 2.75 (95% confidence interval, 1.01 to 7.93) and an HLA-DR beta1 identity of 0.2 (95% confidence interval, 0.06 to 0.99) for suffering an acute rejection episode. In addition, the serum creatinine at discharge was higher in PlA2-positive versus PlA2-negative patients (2.2 +/- 1.6 versus 1.5 +/- 0.6 mg/dl, respectively; P = 0.01), and the prevalence of proteinuria >1.5 g/d 1 yr after transplantation was significantly higher among patients showing the PlA2 allele (16% versus 3%; P = 0.02). Finally, in the entire cohort of patients, the 2-yr graft survival was significantly lower in PlA2-positive (n = 43) compared with PlA2-negative (n = 76) patients (85.7% versus 97.2%; P = 0.015). No differences were found in patient survival (95.2% versus 98.7%, respectively). Proportional hazards regression analysis (Cox regression model) confirmed that serum creatinine level at discharge is the best predictor of allograft survival, followed by CMV status, delayed graft function, and the glycoprotein IIIa/IIb genotype. The PlA2 polymorphism is an independent risk factor for acute renal graft rejection, affecting short-term graft survival. Future studies aimed at preventing the hemostatic imbalance favoring platelet aggregation associated with this polymorphism may be important in preventing acute rejection and its impact on chronic rejection.
PDF
