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Platelet Storage Pool Defect
Platelet storage pool deficiency is a type of coagulopathy characterized by defects in the granules in platelets, particularly a lack of granular non-metabolic adenosine diphosphate. Individuals with adenosine diphosphate deficient ''storage pool disease'' present a prolonged bleeding time due to impaired aggregation response to fibrillar collagen. Symptoms and signs The ''presentation'' (signs/symptoms) of an individual with platelet storage pool deficiency is as follows: :::::::*Unusual bleeding(after surgical procedure) :::::::* Anemia :::::::*Decrease mean platelet volume :::::::*Myelodysplasia Cause The condition of platelet storage pool deficiency can be acquired or inherited (genetically passed on from the individuals parents). Some of the causes of platelet storage pool deficiency when acquired are: ::::::::* Hairy-cell leukemia ::::::::* Cardiovascular bypass Mechanism In terms of the pathophysiology of platelet storage pool deficiency one must consider several fa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Coagulopathy
Coagulopathy (also called a bleeding disorder) is a condition in which the blood's ability to coagulate (form clots) is impaired. This condition can cause a tendency toward prolonged or excessive bleeding (bleeding diathesis), which may occur spontaneously or following an injury or medical and dental procedures. Coagulopathies are sometimes erroneously referred to as "clotting disorders", but a clotting disorder is the opposite, defined as a predisposition to excessive clot formation (thrombus), also known as a hypercoagulable state or thrombophilia. Signs and symptoms Coagulopathy may cause uncontrolled internal or external bleeding. Left untreated, uncontrolled bleeding may cause damage to joints, muscles, or internal organs and may be life-threatening. People should seek immediate medical care for serious symptoms, including heavy external bleeding, blood in the urine or stool, double vision, severe head or neck pain, repeated vomiting, difficulty walking, convulsions, or se ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Flow Cytometry
Flow cytometry (FC) is a technique used to detect and measure physical and chemical characteristics of a population of cells or particles. In this process, a sample containing cells or particles is suspended in a fluid and injected into the flow cytometer instrument. The sample is focused to ideally flow one cell at a time through a laser beam, where the light scattered is characteristic to the cells and their components. Cells are often labeled with fluorescent markers so light is absorbed and then emitted in a band of wavelengths. Tens of thousands of cells can be quickly examined and the data gathered are processed by a computer. Flow cytometry is routinely used in basic research, clinical practice, and clinical trials. Uses for flow cytometry include: * Cell counting * Cell sorting * Determining cell characteristics and function * Detecting microorganisms * Biomarker detection * Protein engineering detection * Diagnosis of health disorders such as blood cancers * Measuring ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hypocoagulability
In medicine (hematology), bleeding diathesis is an unusual susceptibility to bleed (hemorrhage) mostly due to hypocoagulability (a condition of irregular and slow blood clotting), in turn caused by a coagulopathy (a defect in the system of coagulation). Therefore, this may result in the reduction of platelets being produced and leads to excessive bleeding. Several types of coagulopathy are distinguished, ranging from mild to lethal. Coagulopathy can be caused by thinning of the skin (Cushing's syndrome), such that the skin is weakened and is bruised easily and frequently without any trauma or injury to the body. Also, coagulopathy can be contributed by impaired wound healing or impaired clot formation. Signs and symptoms Complications Following are some complications of coagulopathies, some of them caused by their treatments: Causes While there are several possible causes, they generally result in excessive bleeding and a lack of clotting. Acquired Acquired causes of coagu ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Antifibrinolytic
Antifibrinolytics are a class of medication that are inhibitors of fibrinolysis. Examples include aminocaproic acid (ε-aminocaproic acid) and tranexamic acid Tranexamic acid (TXA) is a medication used to treat or prevent excessive blood loss from major trauma, postpartum bleeding, surgery, tooth removal, nosebleeds, and heavy menstruation. It is also used for hereditary angioedema. It is taken eit .... These lysine-like drugs interfere with the formation of the fibrinolytic enzyme plasmin from its precursor plasminogen by plasminogen activators (primarily t-PA and u-PA) which takes place mainly in lysine rich areas on the surface of fibrin. References External links * * {{Antihemorrhagics Transfusion medicine ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Chédiak–Higashi Syndrome
Chédiak–Higashi syndrome (CHS) is a rare autosomal recessive disorder that arises from a mutation of a lysosomal trafficking regulator protein, which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections, albinism, and peripheral neuropathy. In Chédiak–Higashi syndrome, the lysosomal trafficking regulator (LYST) gene is mutated, leading to disruption of protein synthesis as well as the storage and secretory function of lysosomal granules in white blood cells. This results in defective white blood cell function with enlarged vesicles. This syndrome also leads to neutropenia and phagocyte bactericidal dysfunction due to impaired chemotaxis. Deficiency in serotonin and adenosine-phosphate-containing granules in platelets causes impaired platelet aggregation, leading to prolonged bleeding time. Thus, patients are susceptible to infections and often present with oculo-cutaneous albinism and coagulation defects. Patients often p ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hermansky–Pudlak Syndrome
Heřmanský–Pudlák syndrome (often written Hermansky–Pudlak syndrome or abbreviated HPS) is an extremely rare autosomal recessive disorder which results in oculocutaneous albinism (decreased pigmentation), bleeding problems due to a platelet abnormality ( platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). It is considered to affect around 1 in 500,000 people worldwide, with a significantly higher occurrence in Puerto Ricans, with a prevalence of 1 in 1800. Many of the clinical research studies on the disease have been conducted in Puerto Rico. There are eight classic forms of the disorder, based on the genetic mutation from which the disorder stems. Signs and symptoms There are three main disorders caused by Hermansky–Pudlak syndrome, which result in these symptoms: * Albinism and eye problems: Individuals will have varying amounts of skin pigment (melanin). Because of the albinism there are eye pro ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Dense Granule
Dense granules (also known as dense bodies or delta granules) are specialized secretory organelles. Dense granules are found only in platelets and are smaller than alpha granules.Michelson, A. D. (2013). ''Platelets'' (Vol. 3rd ed). Amsterdam: Academic Press. The origin of these dense granules is still unknown, however, it is thought that may come from the mechanism involving the endocytotic pathway.Ambrosio, A. L., Boyle, J. A., & Di Pietro, S. M. (2012). Mechanism of platelet dense granule biogenesis: study of cargo transport and function of Rab32 and Rab38 in a model system. ''Blood'', ''120''(19), 4072–4081. doi:10.1182/blood-2012-04-420745 Dense granules are a sub group of lysosome-related organelles (LRO). There are about three to eight of these in a normal human platelet.McNicol, A., & Israels, S. J. (1999). ''Platelet dense granules: Structure, function and implications for haemostasis'' doi://doi.org/10.1016/S0049-3848(99)00015-8 " In unicellular organisms They are found ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Quebec Platelet Disorder
Quebec platelet disorder (QPD) is a rare autosomal dominant bleeding disorder first described in a family from the province of Quebec in Canada. The disorder is characterized by large amounts of the fibrinolytic enzyme urokinase-type plasminogen activator (uPA) in platelets. This causes accelerated fibrinolysis (blood clot breakdown) which can result in bleeding. Presentation Individuals with QPD are at risk for experiencing a number of bleeding symptoms, including joint bleeds, hematuria, and large bruising. Pathophysiology The disorder is characterized by large amounts of uPA in platelets.Kahr, 2001 Consequently, stored platelet plasminogen is converted to plasmin, which is thought to play a role in degrading a number of proteins stored in platelet α-granules. These proteins include platelet factor V, von Willebrand factor, fibrinogen, Thrombospondin 1, thrombospondin-1, and osteonectin. There is also a quantitative deficiency in the platelet protein multimerin 1 (MMRN1). Furt ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gray Platelet Syndrome
Gray platelet syndrome (GPS), or platelet alpha-granule deficiency, is a rare congenital autosomal recessive 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. The name derives from the initial observation of gray appearance of platelets with a paucity of granules on blood films from a patient with a lifelong bleeding disorder. Signs and symptoms Signs of GPS typically arise at birth or in childhood, these signs and symptoms include thrombocytopenia, bruising susceptibility, and epistaxis. Typically, the observed low platelet count in individuals is progressive, this can result in fatal hemorrhages later in life. Additionally, females who are affected may experience irregular menstrual cycles and heavy menstrual bleeding. Another common effect of GPS is myelofibrosis, where scar tissue builds up in the bone marrow causing it to be unable to ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Platelet Alpha-granule
Alpha granules, (α-granules) also known as platelet alpha-granules are a cellular component of platelets. Platelets contain different types of granules that perform different functions, and include alpha granules, dense granules, and lysosomes. Of these, alpha granules are the most common, making up between 50% to 80% of the secretory granules. Alpha granules contain several growth factors. Contents Contents include insulin-like growth factor 1, platelet-derived growth factors, TGF beta, platelet factor 4 (which is a heparin-binding chemokine) and other clotting proteins (such as thrombospondin, fibronectin, factor V, and von Willebrand factor). The alpha granules express the adhesion molecule P-selectin and CD63. These are transferred to the membrane after synthesis. The other type of granules within platelets are called dense granules. Clinical significance A deficiency of alpha granules is known as gray platelet syndrome Gray platelet syndrome (GPS), or platelet alpha-gra ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
