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Purpura fulminans may also lead to severe large vessel venous thrombosis if untreated in its early stages. [2] Purpura fulminans secondary to severe infection is self-limiting. [2] In cases of homozygous protein C deficiency, episodes of purpura fulminans and other thrombotic events are recurrent. [4]
Manifestation of purpura fulminans as it is usually associated with reduced protein C plasma concentrations of <5 mg IU/dL. [7] The normal concentration of plasma protein C is 70 nM (4 μg/mL) with a half live of approximately 8 hours. [ 2 ]
Protein C, also known as autoprothrombin IIA and blood coagulation factor XIV, [5]: 6822 [6] is a zymogen, that is, an inactive enzyme.The activated form plays an important role in regulating anticoagulation, inflammation, and cell death and maintaining the permeability of blood vessel walls in humans and other animals.
The condition is related to purpura fulminans, a complication in infants with sepsis which also involves skin necrosis. These infants often have protein C deficiency as well. There have also been cases in patients with other deficiency, including protein S deficiency, [6] [7] activated protein C resistance (Factor V Leiden) [8] and antithrombin ...
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.
Protein C Anticoagulant Pathway: Thrombin escaping from a site of vascular injury binds to its receptor thrombomodulin (TM) on the intact cell surface. As a result, thrombin loses its procoagulant properties and instead becomes a potent activator of protein C. Activated protein C (APC) functions as a circulating anticoagulant, which ...
Palpable purpura , petechiae ... in small children or babies may instead be the life-threatening purpura fulminans, ... antibodies, CRP = C-reactive protein, ...
Purpura (/ ˈ p ɜːr p jʊər ə / [1]) is a condition of red or purple discolored spots on the skin that do not blanch on applying pressure. The spots are caused by bleeding underneath the skin secondary to platelet disorders, vascular disorders, coagulation disorders, or other causes. [ 2 ]