Transient Visual Loss Triggered by Scuba Diving in a Patient with a Petrous Epidermoid and Combined Thrombotic Risk Factors

Ruíz‐Argüelles

et al. 2014

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.

Section: The Discovery Of Sps: From Its Initial Description To Full Cmentioning

confidence: 99%

Section: The Discovery Of Sps: From Its Initial Description To Full Cmentioning

confidence: 99%

Sticky Platelet Syndrome: History and Future Perspectives

Ruíz‐Argüelles

et al. 2014

“…The recurrence of thrombosis during adequate anticoagulation therapy with low-molecular-weight-heparin (LMWH) or vitamin K antagonists (VKA) is an interesting clinical feature, reported by several authors. 14,23,27,28 The patient with initial venous thrombosis or pulmonary embolism (PE), under efficient anticoagulation treatment (e.g., therapeutic prolongation of prothrombin time in case of VKA) and suffering from the arterial or new venous thrombosis in a short time (few months) after the first event, represents a typical scenario. The inability of conventional anticoagulation drugs to directly inhibit platelet functions is regarded as a cause.…”

Section: Clinical Manifestation and Diagnosis Of Sticky Platelet Syndmentioning

confidence: 99%

Sticky Platelet Syndrome

Holly

2013

Sticky platelet syndrome (SPS) is a thrombophilic thrombocytopathy with familial occurrence and autosomal dominant trait, characterized by an increased in vitro platelet aggregation in response to low concentrations of adenosine diphosphate (ADP) and/or epinephrine (EPI). According to aggregation pattern, three types of the syndrome can be identified (hyperresponse after both reagents, Type I; EPI alone, Type II; ADP alone, Type III). Clinically, the syndrome is associated with both venous and arterial thrombosis. In pregnant women, complications such as fetal growth retardation and fetal loss have been reported. The first thrombotic event usually occurs before 40 years of age and without prominent acquired risk factors. Antiplatelet drugs generally represent adequate treatment. The use of other antithrombotics is usually ineffective and may result in the recurrence of thrombosis. In most patients, low doses of antiplatelet drugs (acetylsalicylic acid, 80-100 mg/d) lead to normalization of hyperaggregability. Combination of SPS with other thrombophilic disorders has been described. Despite several studies investigating platelet glycoproteins' role in platelets' activation and aggregation, the precise defect responsible for the syndrome remains unknown. The aim of this review is to summarize authors' own experience about SPS and the clinical data indexed in selected databases of medical literature (PubMed and Scopus).

“…After the first such report by Chaturvedi and Dzieczkowski in 1999 of a young woman with recurrent stroke, protein S deficiency, activated protein C resistance (APCR) caused by heterozygous factor V Leiden, and SPS, 24 other cases of combined thrombophilia (with elevated FVIII activity, antiphospholipid syndrome, prothrombin G20201A polymorphism, hyperhomocysteinemia) were published. 21,25,26,32,34,40 Of note, a larger study by Ruiz-Argüelles et al found combined thrombophilia in the majority of patients with platelet hyperagregability. 28…”

Section: Further Clinical and Laboratory Characterization Of Sticky Pmentioning

confidence: 99%

“…Further evidence for a good efficacy of ASA, although mostly in a limited number of patients, was provided by other authors in later years. [18][19][20][21][22][23][24][28][29][30][31][32][33][34][35][36][37][38][39][40] Data on other antiplatelet agents are scarce.…”

Section: Treatment Approach To Sticky Platelet Syndromementioning

confidence: 99%

Sticky Platelet Syndrome: 35 Years of Growing Evidence

Holly

2019

Since the identification of antithrombin deficiency by Egeberg in 1956, ongoing research in prothrombotic defects continues to progress. Interestingly, past research has predominantly focused on coagulation factors and not on other components of the hemostatic system. The possible role of platelet function defects in the development of thrombotic events was suggested for the first time in the late 1970s, when an increased platelet adhesiveness and aggregation after epinephrine (EPI) and adenosine diphosphate (ADP) was found in a group of patients with unexplained transient ischemic attack. Clinical evidence for other types of thrombotic events (e. g. myocardial infarction, ischemic stroke, optic neuropathy, and pregnancy-related complications) with similar laboratory findings was provided by several authors in the 1980s and 1990s, with Drs. Mammen and Bick undertaking key research. The familial occurrence was noted as well, and the term sticky platelet syndrome was introduced by Holliday in 1983 to describe the defect. The term in our present understanding describes a thrombophilic qualitative platelet disorder characterized by increased in vitro platelet aggregation after the addition of very low concentrations of ADP and/or EPI and an increased risk of thromboembolic (predominantly arterial) events. Although now recognized for 35 years, significant issues, namely its etiology, inheritance, epidemiology, and diagnostics, remain a matter of vigorous debate. The aim of this review is to summarize the history, key works, and present understanding of the syndrome and to outline present-day diagnostic and clinical problems and controversies.