Coagulation disorder

Introduction Coagulopathy refers to abnormality of one or more coagulation factors such as congenital or acquired coagulation factor deficiency, vascular wall damage, platelet dysfunction, lack or increase of anticoagulant substances, and excessive activation of fibrinolytic system can lead to blood coagulation obstacle. Congenital coagulopathy: hemophilia A, B, C; von Willebrand's disease (VWD, von Willebrand's disease); coagulation factor deficiency disease (the following coagulation factors are congenital deficiency Symptoms - I, II, V, VII, X, XI, XII, XIII, kallikrein). Acquired clotting factor deficiency: vitamin K deficiency; liver disease; anticoagulant therapy; DIC; acute primary fibrinolysis; large infusion of stock blood; clotting factor inhibitory substance.

Cause

1. Congenital or acquired clotting factor deficiency, vascular wall damage, and platelet dysfunction.

2. Lack or increase of anticoagulant substances and excessive activation of fibrinolytic system.

Normal human blood is in a liquid state in blood vessels. When the blood vessel is damaged, the blood flows out of the blood vessel and is blocked into a blockage at the broken part, so that the bleeding stops. The hemostasis mechanism occurs due to the activation of the coagulation system, that is, a series of biochemical processes occur in the blood coagulation factors, which ultimately leads to the formation of fibrin clots. At the same time, there are a series of anti-coagulation factors and fibrinolytic systems in the plasma, which can effectively prevent excessive blood coagulation and re-dissolve the formed clots to re-open the blood vessel cavity. These mechanisms are interconnected and restricted in the body, thus ensuring the normal physiological functions of the body. Therefore, although a small amount of fibrin is formed in the blood vessel at normal time, the blood flow state is maintained due to continuous dissolution. Once the balance changes, various pathological phenomena will occur.

Naturally occurring physiological anticoagulants include antithrombin III, protein C, heparin, 2 macroglobulin, 1 antitrypsin, 2 anti-plasmin, and the like. Its congenital deficiency can produce a high tendency to thrombosis. Too much physiological anticoagulant is rare. Increased pathological anticoagulant substances are found in the body when the body produces specific antibodies to a certain coagulation factor (acquired), which can lead to coagulopathy.

an examination

Related inspection

Ethanol gel test simple thrombin generation test plasma kallikrein assay simple thrombin generation test coagulation factor activity assay (factor VIII: C, IX: C)

1. Carefully ask about the medical history.

2. Check the coagulation function and compare it with the relevant normal values.

It is divided into two categories: congenital and acquired. Congenital coagulopathy: hemophilia A, B, C; von Willebrand's disease (VWD, von Willebrand's disease); coagulation factor deficiency disease (the following coagulation factors are congenital deficiency Symptoms - I, II, V, VII, X, XI, XII, XIII, kallikrein). Acquired clotting factor deficiency: vitamin K deficiency; liver disease; anticoagulant therapy; DIC; acute primary fibrinolysis; large infusion of stock blood; clotting factor inhibitory substance.

Clinical manifestations Hemorrhage caused by congenital coagulation factor deficiency such as hemophilia mainly manifests as traumatic or postoperative bleeding, skin and mucous membrane bleeding, severe muscle and joint hemorrhage, forming a single deep hematoma. Acquired coagulation factor deficiency, often lack of joint factors, bleeding with nasal discharge, gums, skin, digestive tract, urinary tract bleeding, can also be muscle hematoma, joint or intracranial hemorrhage is rare. With clinical manifestations of primary disease, and thrombocytopenia, vascular wall dysfunction or hyperfibrinolysis.

Differential diagnosis

The diagnosis should be differentiated from the following symptoms:

1. Disorders of clotting factor function: Coagulopathy is a hemorrhagic disease caused by clotting factor deficiency or dysfunction, which can be divided into two major categories: hereditary and acquired. Hereditary coagulopathy is generally a single clotting factor deficiency, mostly bleeding symptoms in infants and young children, mainly family history; acquired coagulopathy is more common, rehabilitation patients often have multiple clotting factor deficiency, mostly in adulthood Clinically, in addition to bleeding, there are symptoms and signs of primary disease.

2. Coagulation dysfunction: Coagulopathy is a hemorrhagic disease caused by a deficiency or dysfunction of coagulation factors, which can be divided into two major categories: hereditary and acquired. Hereditary coagulopathy is generally a single clotting factor deficiency, mostly bleeding symptoms in infants and young children, mainly family history; acquired coagulopathy is more common, rehabilitation patients often have multiple clotting factor deficiency, mostly in adulthood Clinically, in addition to bleeding, there are symptoms and signs of primary disease.

3. Intravascular coagulation: seen in disseminated intravascular coagulation, also known as DIC, is caused by various causes of small blood vessels coagulation, the formation of a wide range of microthrombus, a large number of coagulation factors are consumed, and secondary activation of fibrinolysis, resulting in Severe extensive systemic bleeding. The disease is also known as: defibrin syndrome, consumptive coagulopathy or intravascular coagulation-defibrinolytic syndrome.

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