Sticky platelet syndrome
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Sticky platelet syndrome (SPS) is a heritable disorder of platelet function in which platelet hyperaggregation leads to hypercoagulability.{{cite journal | vauthors = Mammen EF | title = Sticky platelet syndrome | journal = Seminars in Thrombosis and Hemostasis | volume = 25 | issue = 4 | pages = 361–365 | year = 1999 | pmid = 10548069 | doi = 10.1055/s-2007-994939 | doi-access = free }}{{cite journal | vauthors = Frenkel EP, Mammen EF | title = Sticky platelet syndrome and thrombocythemia | journal = Hematology/Oncology Clinics of North America | volume = 17 | issue = 1 | pages = 63–83 | date = February 2003 | pmid = 12627663 | doi = 10.1016/S0889-8588(02)00096-5 }}{{cite journal | vauthors = Mears KA, Van Stavern GP | title = Bilateral simultaneous anterior ischaemic optic neuropathy associated with Sticky Platelet Syndrome | journal = The British Journal of Ophthalmology | volume = 93 | issue = 7 | pages = 885–6, 913 | date = July 2009 | pmid = 18662911 | doi = 10.1136/bjo.2008.142919 | s2cid = 42202617 }}{{cite journal | vauthors = Mammen EF, Barnhart MI, Selik NR, Gilroy J, Klepach GL | title = "Sticky platelet syndrome": a congenital platelet abnormality predisposing to thrombosis? | journal = Folia Haematologica | volume = 115 | issue = 3 | pages = 361–365 | year = 1988 | pmid = 2465231 }}{{cite journal | vauthors = Mühlfeld AS, Ketteler M, Schwamborn K, Eitner F, Schneider B, Gladziwa U, Knüchel R, Floege J | display-authors = 6 | title = Sticky platelet syndrome: an underrecognized cause of graft dysfunction and thromboembolic complications in renal transplant recipients | journal = American Journal of Transplantation | volume = 7 | issue = 7 | pages = 1865–1868 | date = July 2007 | pmid = 17532753 | doi = 10.1111/j.1600-6143.2007.01835.x | doi-access = free }} It was first described by Mammen in 1983.{{Cite book| vauthors = Bick RL |title=Hematological complications in obstetrics, pregnancy, and gynecology |publisher=Cambridge University Press |location=Cambridge, UK |year=2006 |pages=147 |isbn=978-0-521-83953-2 }} It is inherited in an autosomal dominant pattern.{{Cite book| vauthors = McKay R, Gambling DR |title=Obstetric anesthesia and uncommon disorders |publisher=Cambridge University Press |location=Cambridge, UK |year=2008 |pages=308 |isbn=978-0-521-87082-5 }} It has not been associated with a specific gene, and it is not recognized as an entity in OMIM.
It can present in conjunction with protein S deficiency and factor V Leiden.{{cite journal | vauthors = Chaturvedi S, Dzieczkowski JS | title = Protein S deficiency, activated protein C resistance and sticky platelet syndrome in a young woman with bilateral strokes | journal = Cerebrovascular Diseases | volume = 9 | issue = 2 | pages = 127–130 | year = 1999 | pmid = 9973658 | doi = 10.1159/000015911 | s2cid = 35754773 }} It is not currently known if sticky platelet syndrome is a distinct condition, or if it represents part of the presentation of a more well characterized coagulation disorder.
SPS has not been widely studied{{cite journal | vauthors = Stasko J, Holly P, Kubisz P | title = A new decade awaits sticky platelet syndrome: where are we now, how do we manage and what are the complications? | journal = Expert Review of Hematology | volume = 15 | issue = 1 | pages = 53–63 | date = January 2022 | pmid = 35034520 | doi = 10.1080/17474086.2022.2030217 | s2cid = 246905075 }} and is not widely known.{{cite journal | vauthors = Solis-Jimenez F, Hinojosa-Heredia H, García-Covarrubias L, Soto-Abraham V, Valdez-Ortiz R | title = Sticky Platelet Syndrome: An Unrecognized Cause of Acute Thrombosis and Graft Loss | journal = Case Reports in Nephrology | volume = 2018 | pages = 3174897 | date = 2018 | pmid = 29850310 | pmc = 5937576 | doi = 10.1155/2018/3174897 | doi-access = free }}
Signs and symptoms
Symptoms are related to hypercoagulability, usually presenting as venous thromboembolisms, arterial thrombosis, myocardial infarction, angina, and stroke.
Cause
Diagnosis
SPS is diagnosed by demonstrating platelet hyperaggregability. In a lab test called aggregometry, platelet stickiness is stimulated with epinephrine (EPI) and/or adenosine diphosphate (ADP).{{cite journal | vauthors = Weber M, Gerdsen F, Gutensohn K, Schoder V, Eifrig B, Hossfeld DK | title = Enhanced platelet aggregation with TRAP-6 and collagen in platelet aggregometry in patients with venous thromboembolism | journal = Thrombosis Research | volume = 107 | issue = 6 | pages = 325–328 | date = September 2002 | pmid = 12565719 | doi = 10.1016/S0049-3848(02)00351-1 }} This test is not possible for patients being treated with acetylsalicylic acid until that substance has sufficiently cleared from their system.{{citation needed|date=March 2019}}
Treatment
Treatment usually consists of a daily low dose (80–100 mg) of aspirin.{{cite journal | vauthors = Mammen EF | title = Sticky platelet syndrome | journal = Seminars in Thrombosis and Hemostasis | volume = 25 | issue = 4 | pages = 361–365 | year = 1999 | pmid = 10548069 | doi = 10.1055/s-2007-994939 | doi-access = free }} Anticoagulants (e.g. warfarin) or antiplatelets (clopidogrel) are often additionally prescribed following formation of a medically significant clot. Thrombelastography is more commonly being used to diagnose hypercoagulability and monitor anti-platelet therapy.{{citation needed|date=March 2019}}
References
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{{cite journal | vauthors = Bick RL | title = Hereditary and acquired thrombophilic disorders | journal = Clinical and Applied Thrombosis/Hemostasis | volume = 12 | issue = 2 | pages = 125–135 | date = April 2006 | pmid = 16708115 | doi = 10.1177/107602960601200201 | s2cid = 42478313 | citeseerx = 10.1.1.486.6752 }}
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{{Diseases of megakaryocytes|us=y}}