ABSTRACT
Introducción: Se ha demostrado la participación de moléculas de adhesión, tanto en eritrocitos con hemoglobina SS, como en el endotelio vascular; así como factores plasmáticos en el fenómeno de vasoclusión en la drepanocitosis. La banda 3 se refiere a una familia de intercambiadores aniónicos presentes en la membrana de todas las células y organelos celulares, las que bajo ciertas condiciones se agregan en la superficie del eritrocito y son reconocidos por anticuerpos naturales como parte del mecanismo de eliminación de eritrocitos senescentes u oxidados. Objetivo: Evaluar la posible participación de los anticuerpos naturales antibanda 3 en el fenómeno oclusivo en la drepanocitosis. Métodos: Se realizó la determinación seriada de anticuerpos naturales antibanda 3 en 19 enfermos con drepanocitosis en diferentes estadios clínicos mediante un ensayo inmunoenzimático en microplacas acopladas con la proteína banda 3. Resultados: Se demostró una disminución significativa (p < 0.01) de anticuerpos naturales antibanda 3 en las muestras de los enfermos obtenidas en las fases de crisis vasoclusiva dolorosa, disminución que pudiera estar relacionada con un consumo elevado de estos en el proceso de eliminación de eritrocitos SS oxidados de la circulación sanguínea. Las muestras en estado basal se mantuvieron dentro de los límites normales. Conclusión: Los resultados sugieren la participación de los anticuerpos naturales antibanda 3 como elemento regulador de la no adhesión de los eritrocitos SS al endotelio vascular en esta enfermedad(AU)
Introduction : The involvement of adhesion molecules in erythrocytes with hemoglobin SS, in vascular endothelium and also plasma factors have been shown in the vasoocclusion phenomenon in sickle cell anemia (SCA). Band 3 refers to a family of anion exchangers present in the membrane of all cells and cellular organelles which under certain conditions cluster on the erythrocyte surface being recognized by natural antibodies as part of the mechanism of removal of senescent or oxidized erythrocytes. Objective : To evaluate the possible participation of natural anti band 3 antibodies in the occlusive phenomenon in SCA. Methods : A follow up study to determine the presence of natural band 3 antibodies in 19 patients with SCA in different clinical stages was performed using an enzyme immunoassay in microplates coupled with the band 3 protein. Results : Significant low levels (p<0.01) of natural band 3 antibodies were demonstrated in the samples of patients obtained in painful vasoocclusive crisis stages, decrease which may be related to a high consumption of antibodies in the process of removing oxidized SS erythrocytes from blood circulation. Steady state samples were within normal range. Conclusion: The results suggest the involvement of natural band 3 antibodies in the regulation of the adherence of SS erythrocytes to vascular endothelium in SCA(AU)
Subject(s)
Humans , Male , Female , Antibodies , Sickle Cell Trait/immunology , Serial Cross-Sectional StudiesABSTRACT
Immunoglobulins IgG, IgA and IgM were estimated in 20 cases of sickle cell disease in steady state, eleven cases of sickle cell disease in crisis and/or infection, 20 cases of sickle cell trait and 14 normal healthy controls. Immunoglobulin deficiency has not been observed in sickle cell disease. Significantly raised IgG but normal IgA and IgM found in sickle cell disease cases was probably due to chronic stimulation of reticuloendothelial cells secondary to chronic hemolysis. IgM levels were significantly higher in patients with splenic enlargement > 3 cm. There was no alteration in immunoglobulin levels in sickle cell trait.
Subject(s)
Adult , Anemia, Sickle Cell/immunology , Female , Humans , Immunoglobulins/blood , Male , Sickle Cell Trait/immunologyABSTRACT
Sickle cell anaemia, a haemoglobinopathy with serious impacts represents a health challenge in the developing world. Patients are particularly susceptible to repeated infections that may endanger their lives. In this study 83 adults and 30 children were included. Cross-sectionally, serum immunoglobulins [Ig[G], Ig[A] and Ig[M]] and complement factors [C[3]and C[4]] were assessed. In addition prospectively all subjects were immunized by Hepatitis [B] vaccine [Recombinant Hepatitis [B] virus surface antigen] and the immune response was evaluated and followed up by antibodies to the selected antigen. Only serum Ig[A] was elevated in all sickle cell disease and sickle cell trait groups when compared to age matched healthy relatives and Serum Ig[M] was decreased only in sickle cell disease children group. The sickler groups showed close numbers of responders compared to controls in adult groups. The results denoted a possible quantitative difference in the level of Hepatitis [B] virus surface antibody. Further studies to delineate the implicated mechanisms in the immunodeficiency are suggested. Chemoprophylaxis and immunoprophylaxis are highly recommended, meanwhile