Sticky platelet syndrome
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Sticky platelet syndrome (SPS) is a heritable disorder of
platelet Platelets, also called thrombocytes (from Greek θρόμβος, "clot" and κύτος, "cell"), are a component of blood whose function (along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping, thereby i ...
function in which platelet hyperaggregation leads to
hypercoagulability Thrombophilia (sometimes called hypercoagulability or a prothrombotic state) is an abnormality of blood coagulation that increases the risk of thrombosis (blood clots in blood vessels). Such abnormalities can be identified in 50% of people who ...
. It was first described by Mammen in 1983. It is inherited in an autosomal dominant pattern. It has not been associated with a specific gene, and it is not recognized as an entity in
OMIM Online Mendelian Inheritance in Man (OMIM) is a continuously updated catalog of human genes and genetic disorders and traits, with a particular focus on the gene-phenotype relationship. , approximately 9,000 of the over 25,000 entries in OMIM ...
. It can present in conjunction with
protein S deficiency Protein S deficiency is a disorder associated with increased risk of venous thrombosis. Protein S, a vitamin K-dependent physiological anticoagulant, acts as a nonenzymatic cofactor to activate protein C in the degradation of factor Va and factor V ...
and
factor V Leiden Factor V Leiden (rs6025 or ''F5'' p.R506Q) is a variant (mutated form) of human factor V (one of several substances that helps blood clot), which causes an increase in blood clotting (hypercoagulability). Due to this mutation, protein C, an anticoa ...
. 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 and is not widely known.


Signs and symptoms

Symptoms are related to hypercoagulability, usually presenting as venous thromboembolisms, arterial thrombosis,
myocardial infarction A myocardial infarction (MI), commonly known as a heart attack, occurs when blood flow decreases or stops to the coronary artery of the heart, causing damage to the heart muscle. The most common symptom is chest pain or discomfort which may ...
, 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). This test is not possible for patients being treated with
acetylsalicylic acid Aspirin, also known as acetylsalicylic acid (ASA), is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce pain, fever, and/or inflammation, and as an antithrombotic. Specific inflammatory conditions which aspirin is used to treat ...
until that substance has sufficiently cleared from their system.


Treatment

Treatment usually consists of a daily low dose (80–100 mg) of
aspirin Aspirin, also known as acetylsalicylic acid (ASA), is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce pain, fever, and/or inflammation, and as an antithrombotic. Specific inflammatory conditions which aspirin is used to treat inc ...
. Anticoagulants (e.g.
warfarin Warfarin, sold under the brand name Coumadin among others, is a medication that is used as an anticoagulant (blood thinner). It is commonly used to prevent blood clots such as deep vein thrombosis and pulmonary embolism, and to prevent st ...
) or
antiplatelets An antiplatelet drug (antiaggregant), also known as a platelet agglutination inhibitor or platelet aggregation inhibitor, is a member of a class of pharmaceuticals that decrease platelet aggregation and inhibit thrombus formation. They are effecti ...
(
clopidogrel Clopidogrel — sold under the brand name Plavix, among others — is an antiplatelet medication used to reduce the risk of heart disease and stroke in those at high risk. It is also used together with aspirin in heart attacks and following ...
) are often additionally prescribed following formation of a medically significant clot.
Thrombelastography Thromboelastography (TEG) is a method of testing the efficiency of blood coagulation. It is a test mainly used in surgery and anesthesiology, although increasingly used in resuscitations in Emergency Departments, intensive care units, and labor an ...
is more commonly being used to diagnose hypercoagulability and monitor anti-platelet therapy.


References

{{Diseases of megakaryocytes, us=y Coagulopathies Syndromes affecting blood