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

The syndrome is believed to be hereditary.

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}}

Category:Coagulopathies

Category:Syndromes affecting blood