Storage pool deficiencies are a group of bleeding disorders caused by problems with platelet granules . Granules are little sacs inside the platelet where proteins and other chemicals are stored. Platelets are used by our bodies to create something like an internal bandage (a temporary patch) to stop bleeding.
What is storage pool disease?
Storage pool deficiencies are a group of bleeding disorders caused by problems with platelet granules . Granules are little sacs inside the platelet where proteins and other chemicals are stored. Platelets are used by our bodies to create something like an internal bandage (a temporary patch) to stop bleeding.
What is delta storage pool deficiency?
Introduction. Delta storage pool deficiency (δ-SPD) is a rare group of platelet disorders characterized by a reduction in the number or content of dense granules . δ-SPD causes a mild to moderate bleeding diathesis characterized mainly by mucocutaneous bleeding.
What is platelet function disorder?
Platelet function disorders are a group of bleeding disorders in which the platelets do not function appropriately, leading to bleeding . These disorders can be inherited (passed down in families) or acquired (developed after birth).
What is platelet dense granule deficiency?
Dense Granule Deficiency
Platelets are normal in size and show unremarkable ultrastructural features, except for a marked decline or absence of dense granules. Patients demonstrate a mild to moderate bleeding diathesis such as easy bruising, epistaxis, gingival bleeding, and menorrhagia.
Is storage pool disease inherited?
Platelet storage pool deficiency refers to a group of conditions that can be acquired (non- inherited ) or inherited. Hereditary forms of the condition may be inherited in an autosomal dominant , autosomal recessive , or X-linked manner.
What is GREY platelet syndrome?
Gray platelet syndrome is a bleeding disorder associated with abnormal platelets , which are small blood cells involved in blood clotting. People with this condition tend to bruise easily and have an increased risk of nosebleeds (epistaxis).
What is Scott syndrome?
Scott syndrome is an extremely rare bleeding disorder that confirms the essential role of these anionic procoagulant phospholipids. In Scott patients, phosphatidylserine externalization and microparticle shedding are dramatically impaired.
What is Glanzmann disease?
Glanzmann thrombasthenia (GT) is a rare inherited blood clotting (coagulation) disorder characterized by the impaired function of specialized cells (platelets) that are essential for proper blood clotting. Symptoms of this disorder usually include abnormal bleeding, which may be severe.
What does Thrombasthenia mean?
The term thrombasthenia means weak platelets . Glanzmann thrombasthenia (GT) is one of several inherited disorders of platelet function, which also include Bernard-Soulier syndrome, as well as deficiencies of platelet adhesion, aggregation, and secretion.
How is platelet dysfunction treated?
Treatment of Platelet Dysfunction
If platelet dysfunction is caused by a drug, stopping the drug usually is the only treatment needed . People with an inherited platelet disorder that causes excessive bleeding usually should not take drugs that impair platelet function.
What diseases are caused by low platelets?
- Have a blood disorder that affects your bone marrow, called aplastic anemia.
- Have cancer such as leukemia or lymphoma, which damages your bone marrow.
- Have a platelet-lowering disease like Wiskott-Aldrich or May-Hegglin syndromes.
- Have a virus such as chickenpox, mumps, rubella, HIV, or Epstein-Barr.
What causes platelet disorders?
A large number of drugs may cause thrombocytopenia Drug-induced immunologic destruction Platelet destruction can develop because of immunologic causes ( viral infection , drugs, connective tissue or lymphoproliferative disorders, blood transfusions) or nonimmunologic causes (sepsis... read more , typically by triggering ...
What does platelet activating factor do?
Platelet-activating factor (PAF) is a potent phospholipid mediator that was first described by its ability to cause platelet aggregation and dilation of blood vessels . Now it is also known as a potent mediator of inflammation, allergic responses, and shock.
How much does platelet transfusion increase platelets?
Platelet dosage
A platelet concentrate containing approximately 0.7 X 10 11 platelets should cause a platelet count increase of 5000 to 10,000 / μl in an average sized adult.
How many dense granules on the average are contained in a platelet?
Platelet dense granules are the smallest granules (~150 nm) and appear as dense bodies on electron microscopy (see Figure 2.1), due to their high calcium and phosphate content [9]. There are about 3–8 dense granules per platelet. In addition they contain high concentrations of adenine nucleotides and serotonin.
