2013, Number 3
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Rev Hematol Mex 2013; 14 (3)
The Sticky Platelet Syndrome (SPS)
Césarman-Maus G, Villa R, Kubisz P, González-Ramírez M, Ruiz-Delgado GJ, Ruiz-Arguelles GJ
Language: Spanish
References: 34
Page: 149-153
PDF size: 387.32 Kb.
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 concentrations
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.
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Ruiz-Argüelles GJ, Alarcón-Urdaneta C, Calderón-García J, Ruiz-Delgado GJ.: Primary thrombophilia in México VIII: Description of five kindreds of familial sticky platelet syndrome phenotype. Rev Hematol Méx 2011; 12:73-78.
Ruiz-Argüelles GJ, Garcés-Eisele J, Camacho-Alarcón C, Reyes-Nuñez V, Moncada-González B, Valdés-Tapia P, León-Montes N, Ruiz-Delgado GJ. Primary thrombophilia in Mexico IX: The glycoprotein IIIa PLA1/A2 polymorphism is not associated with the sticky platelet syndrome phenotype. Clin Appl Thromb Hemost 2012 Jun 29. [Epub ahead of print]
Moncada B, Ruiz-Argüelles GJ, Castillo-Martínez C.: The sticky platelet syndrome. Hematology. 2013 Feb 19. [Epub ahead of print]