Resumen

SVARCH, Eva. Physiopathologic features of drepanocytemia. Rev Cubana Hematol Inmunol Hemoter [online]. 2009, vol.25, n.1, pp. 0-0. ISSN 1561-2996.

Hemoglobin S (Hb) is the consequence of a point mutation in of b globin chain 6 position resulting in glutamic acid substitution by valine. HB S polymerizes in conditions of oxygen low tension deforming the erythrocyte, the latter with the HB polymerized, has a shorten survival occluding the microcirculation, causing a chronic hemolytic anemia, and painful vasoconstrictive crises noting down to large extent the clinical picture of disease. Vascular occlusion is a complex process due mainly to transit time extension of erythrocytes through microcirculation and/or decrease of delayed time of polymerization of HbS. Nowadays, occlusion in the microcirculation is considered as an injury way of reperfusion in which oxidative stress of coagulation participates in the process. Hemolytic anemia contributes to vascular occlusion because of the reticulocytes, with more adhesive molecules in its surface than the mature ones, to determines its greater adhesion to endothelium, and because of the plasma free Hb to consume nitric oxide (NO). NO acts in different ways, where the most important is the deviation of normal balance between vasodilatation and vasoconstriction. As consequence from these facts we describe a sub-phenotype characterized by pulmonary hypertension, stroke, priapism, and malleolar ulcer. There are genetic factors influencing in clinical picture severity, e.g. 158 C-T mutation in globin gen g, and a-thalassemia. The first one increase the HBF, and the second one decrease the CHCM, the number of thick cells, and the hemolysis identity. However, relation between a-thalassemia and severity of disease is not clear.

Palabras clave : Drepanocytemia; hemolytic anemia; vascular occlusion..

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