RESUMEN
Hace tres décadas Holliday y Mammen describieron a pacientes con enfermedades tromboembólicas arteriales y venosas asociadas con hiperreactividad plaquetaria hereditaria. Ellos llamaron a este fenó- meno protrombótico “síndrome de plaquetas pegajosas” (SPP). El SPP tiene un rasgo autosómico dominante, definido por un aumento en la agregación plaquetaria inducida por diversas concentraciones de dos agonistas plaquetarios –adenosín difosfato (ADP) y/o epinefrina (EPI). Se han identificado tres variantes de esta enfermedad: hiperagregabilidad a ADP y EPI- tipo I, solo a EPI- tipo II, y solo a ADP- tipo III. La hiperagregabilidad se diagnostica por medio de agregometría plaquetaria, aunque exista controversia al hacer el diagnóstico, ya que la concentración de los agonistas no está estandarizada, y no existe un consenso en el porcentaje de agregación plaquetaria. Es importante tomar en cuenta la hiperagregabilidad de los agonistas ADP y EPI, porque se ha relacionado con varias enfermedades adquiridas, como enfermedades metabólicas (diabetes mellitus, aterosclerosis) y enfermedades inflamatorias (sepsis y enfermedades del sistema inmune). A pesar de que se conoce el fenotipo de la enfermedad, el genotipò no ha podido definirse. Los fármacos antiplaquetarios, como la aspirina y el clopidogrel revierten la hiperreactividad plaquetaria de los pacientes con SPP, lo que resulta en disminución de la tasa de retrombosis.
ABSTRACT
Three decades ago Holiday and Mammen described patients with arterial or venous thromboembolic disease associated with inherited platelet hyper reactivity and named this prothrombotic state ̈sticky platelet syndrome ̈ (SPS) . SPS has an autosomal dominant trait, defined by increased platelet aggregation in response to low concen- trations of 2 platelet agonists – adenosine diphosphate (ADP) and / or epinephrine (EPI). There are 3 distinct types (hyperaggregability to ADP and EPI – type I, to EPI alone – type II, to ADP alone – type III), which can be identified. Hyper reactivity is diagnosed by platelet aggregometry, although there has been controversy in diagnosing SPS because the concentration of agonists are not standardized, and there is no consensus on the percent of platelet aggregation that would be considered positive. It is important to bear in mind the platelet hyperaggregability agonists, EPI and ADP, because they have been described in several acquired disorders, such as complex metabolic disease (diabetes mellitus, atherosclerosis)and inflammatory disorders (sepsis, systemic immune diseases).
Despite the fact that the phenotype if the disease is well known, its genotype has not been defined. Antiplatelet drugs, such as aspirin and clopidogrel, have reverted the platelet hyperreactivity of patients with SPS, translating this into a substantial decrease of their re-thrombosis rate.
