Sticky Platelet Syndrome: History and Future Perspectives

Abstract

The sticky platelet syndrome (SPS) is a thrombophilic qualitative platelet disorder with familial occurrence and autosomal dominant trait, characterized by increased in vitro platelet aggregation after low concentrations of adenosine diphosphate and/or epinephrine. Its clinical manifestation includes arterial thrombosis, pregnancy complications (fetal growth retardation and fetal loss), and less often venous thromboembolism. SPS was considered to be a rare thrombophilic disorder, but it can be found relatively often as a cause of unexplained thrombosis, particularly among patients with arterial thrombosis such as stroke. The syndrome was recognized as a distinct disorder in 1983 by Holiday and further characterized in the 1980s and 1990s, with Mammen and Bick providing the key findings. Although recognized for more than 30 years, significant issues, namely the syndrome's etiology, inheritance, and epidemiology, remain unclear. The aim of the first part of this review is to summarize the previous 35 years of the research into, and to provide a brief historical account of, SPS. The history section is focused particularly on the work of two most prominent investigators: Eberhard F. Mammen and Rodger L. Bick. The second part summarizes the present understanding of the syndrome and outlines unresolved issues and the trends in which the future research is likely to continue.

Primary thrombophilia in México XII: Miscarriages are more frequent in persons with the Sticky Platelet Syndrome (SPS)

“…Other authors have also shown increased prevalence of obstetric complications in patients with SPS [ 18 , 19 , 31 ]. For example, our group demonstrated that, in the general Mexican population, the prevalence of SPS and the 677 mutation in the MTHFR gene is 15% and 79%, respectively [ 16 , 17 ].…”

“…However, there is a shortage of literature on this syndrome in the pregnant population. Since the treatment of SPS is simple, cheap, and highly effective, employing aspirin and/or other antiplatelet drugs [ 13 , 16 ], the investigation of SPS in women treated for obstetric complications may be useful and deserves further research. The appropriate use of aspirin and/or other antiplatelet drugs during the pregnancy of patients with SPS could result in the prevention of adverse pregnancy outcomes.…”

“…Sticky platelet syndrome (SPS) was first described by Holliday et al [ 1 ] at the 9 th Conference on Stroke and Cerebral Circulation in Arizona in 1983. Since then, we and others have found that SPS is a common cause of arterial and venous thrombosis [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 ]. SPS is the second most common hereditary thrombophilic condition after resistance to activated protein C and the most common thrombophilia associated with arterial thrombosis, with an incidence of 21% [ 3 , 4 , 5 ].…”

Global assays of hemostasis in the diagnostics of hypercoagulation and evaluation of thrombosis risk

“…This is confirmed by predominance of platelets in such thrombin (so-called ‘white’ thrombi) and by efficiency of antiplatelet agents [ 15 ]. Altered platelet adhesion and aggregation due to increased vWF concentration, decreased ADAMTS13 (a metalloproteinase, that cleaves vWF to smaller, less procoagulant forms), increased in vitro platelet aggregation after low concentrations of adenosine diphosphate and/or epinephrine (sticky platelet syndrome) are associated with arterial thrombosis [ 16 , 17 ].…”

Glycoprotein VI Gene Variants Affect Pregnancy Loss in Patients With Platelet Hyperaggregability

“…5,6 Patients with SPS present with arterial and/or venous thrombosis and fetal loss. 5–12 Although the first patient with SPS was reported in 1983, 13 many physicians remain unfamiliar with this platelet defect and do not consider SPS when screening for thrombophilic risk factors. The diagnosis and classification of SPS relies on platelet aggregometry tests employing appropriate dilutions of 2 platelet aggregation inducers: ADP and EPI.…”