Platelet storage pool deficiency
Encyclopedia
Platelet storage pool deficiency is a type of coagulopathy
characterized by defects in the granules in platelet
s.
It may involve the alpha granules or the dense granules.
Coagulopathy
Coagulopathy is a condition in which the blood’s ability to clot is impaired. This condition can cause prolonged or excessive bleeding, which may occur spontaneously or following an injury or medical and dental procedures.The normal clotting process depends on the interplay of various proteins in...
characterized by defects in the granules in platelet
Platelet
Platelets, or thrombocytes , are small,irregularly shaped clear cell fragments , 2–3 µm in diameter, which are derived from fragmentation of precursor megakaryocytes. The average lifespan of a platelet is normally just 5 to 9 days...
s.
It may involve the alpha granules or the dense granules.
Conditions
Examples include:- Platelet alpha-granulePlatelet alpha-granuleIn platelets, the term "alpha granules" is used to describe granules containing several growth factors.-Contents:Contents include insulin-like growth factor 1, platelet-derived growth factor, TGFβ, platelet factor 4 and other clotting proteins In platelets, the term "alpha granules" is used to...
s- Gray platelet syndromeGray platelet syndromeGray platelet syndrome , or platelet alpha-granule deficiency, is a rare congenital bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets, and the release of proteins normally contained in these granules into the marrow, causing myelofibrosis.GPS is primarily...
- Quebec platelet disorderQuebec Platelet DisorderQuebec Platelet Disorder is a rare, autosomal dominant bleeding disorder described in a family from the province of Quebec in Canada.-Pathophysiology:...
- Gray platelet syndrome
- Dense granuleDense granuleDense granules are specialized secretory organelles.-In unicellular organisms:They are found in animals and in unicellular organisms including Apicomplexa protozoans....
s- Hermansky-Pudlak syndromeHermansky-Pudlak syndromeHermansky–Pudlak syndrome is a rare autosomal recessive disorder which results in oculocutaneous albinism , bleeding problems due to a platelet abnormality , and storage of an abnormal fat-protein compound .There are eight classic forms of the disorder, based on the genetic mutation...
- Chediak-Higashi syndromeChédiak-Higashi syndromeChédiak–Higashi syndrome is a rare autosomal recessive disorder that arises from a microtubule polymerization defect which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections, partial albinism and peripheral neuropathy...
- Hermansky-Pudlak syndrome