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Platelet storage pool deficiency is a family of clotting disorders characterized by deficient granules in platelets. Individuals with these disorders have too few or abnormally functioning alpha granules , delta granules , or both alpha and delta granules and are therefore unable to form effective clots, which leads to prolonged bleeding.
It is associated primarily with pathological bleeding. [5] Hereditary fibrinogen Aα-Chain amyloidosis is a sub-category of congenital dysfibrinogenemia in which the dysfunctional fibrinogen does not cause bleeding or thrombosis but rather gradually accumulates in, and disrupts the function of, the kidney. [6]
The disorder was first described by Egeberg in 1965. [5] The causes of acquired antithrombin deficiency are easier to find than the hereditary deficiency. [3] The prevalence of antithrombin deficiency is estimated at ~0.02 to 0.2% of the general population, and 1-5% of patients with venous thromboembolism. [6]
The following revised diagnostic criteria for essential thrombocythemia were proposed in 2005. [13] The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6. [14] The criteria are as follows: [14] A1. Platelet count > 400 × 10 3 /μL for at least 2 months. A2. Acquired V617F JAK2 ...
Light transmission aggregometry is widely accepted as the gold standard diagnostic tool for assessing platelet function, and a result of absent aggregation with any agonist except ristocetin is highly specific for Glanzmann's thrombasthenia. [9] Following is a table comparing its result with other platelet aggregation disorders:
Factor X deficiency (X as Roman numeral ten) is a bleeding disorder characterized by a lack in the production of factor X (FX), an enzyme protein that causes blood to clot in the coagulation cascade. Produced in the liver FX when activated cleaves prothrombin to generate thrombin in the intrinsic pathway of coagulation.
Each country has its own rules to protect the safety of both donor and recipient. In a typical set of rules, a platelet donor must weigh at least 50 kg (110 lb) and have a platelet count of at least 150 x 10 9 /L (150,000 platelets per mm³). [2] One unit has greater than 3×10 11 platelets. Therefore, it takes 2 liters of blood having a ...
Diagnostic criteria for Wiskott–Aldrich syndrome [19] Mandatory criteria: Low platelets, known as thrombocytopenia (<70,000 platelets/mm3) on two separate tests, AND; Small platelets (platelet volume <7.5 fL) Definitive: Pathogenic WAS variant (or deletion) detected on genetic testing, OR