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Coagulation dysfunction refers to a bleeding disorder caused by a lack of coagulation factors or abnormal function.

Coagulopathy

Definition of coagulopathy

Coagulation dysfunction refers to a bleeding disorder caused by a lack of coagulation factors or abnormal function.

Clinical classification of coagulopathy

Divided into hereditary and acquired two categories.

1. Hereditary coagulation dysfunction is generally a lack of a single coagulation factor, mostly bleeding symptoms in infants and young children, often with a family history.

2. Acquired coagulation dysfunction is more common. Patients often have multiple coagulation factor deficiencies, which mostly occur in adulthood. In addition to clinical bleeding, there are also symptoms and signs of primary disease.

Classification and description of coagulopathy

Coagulopathy

Hemophilia (hemophilia) is the most common group of hereditary coagulation factor deficiency, which can be divided into hemophilia A (factor procoagulant component is lacking) and hemophilia B (factor deficiency). Factor : The biosynthetic genes of C and Factor are located on the X chromosome, so they are called X? Chain diseases, both of which are associated recessive inheritance of the X chromosome. Although female carriers have varying degrees of factor : C or factor activity reduction, they generally have no bleeding symptoms. About one-third of the patients have no family history. It may be due to the fact that there are few males in the family or that they are inherited and ignored, or it may be caused by a genetic mutation.

(A) clinical manifestations

Mainly manifested as bleeding, characterized by bleeding from soft tissues, muscles, and weight-bearing joints. Usually has a tendency to bleed from early childhood, mild can be diagnosed in adolescents or even adults. The earlier the bleeding symptoms appear, the worse the condition becomes. Patients can present with minor trauma or severe bleeding after surgery, often with more than one bleeding during tooth extraction or minor surgery. A few patients have this as their first symptom. Bleeding can last for hours or even weeks. The degree of bleeding is related to plasma factor activity (concentration). Although the factor or activity required for normal hemostasis is 25%, the factor activity is usually less than 5% in those with symptoms. Clinically, hemophilia is classified into severe, medium, light, and subclinical based on factor activity. Bleeding sites are most common in the injured parts of the limbs. Deep tissue hematomas can occur. Those with hematomas can compress nearby nerves such as the femoral nerve, median nerve, and ulnar nerve, causing pain and paralysis. Compression of blood vessels can cause jaundice. Soft tissue bleeding in the neck and throat can suffocate due to obstruction of the airways. There may be abdominal pain in retroperitoneal and mesenteric bleeding. Severe cases can cause nosebleeds, gum bleeding, gastrointestinal bleeding, hematuria, and excessive bleeding can cause anemia. Repeated joint cavity bleeding is seen in critically ill patients, and it usually occurs after minor injuries, and can also spontaneously bleed. There may be local swelling, pain, tenderness, and acute symptoms that last for 3 to 5 days. After the bleeding stops, the blood will gradually be absorbed after several weeks without leaving traces. If it is not absorbed for a long time, it can cause synovitis, repeated bleeding can cause joint stiffness, and eventually lead to permanent joint damage, osteoporosis, limited joint movement, deformation, nearby muscle atrophy, and disability. The most commonly affected joints are ankle joints in infancy and knee joints in children and adults.

The relationship between the deficiency of hemophilia A and B coagulation factors and the degree of bleeding

Hemophilia A and B can develop in the neonatal period, but most of them occur at the age of two. The severity of the former hemorrhage is related to the level of : C activity in its plasma: 0 to 1% of the activity is severe. Patients have spontaneous bleeding, repeated joint bleeding or deep tissue (muscle, internal organs) bleeding from childhood, And often lead to joint deformities; 2% to 5% are medium-sized, patients with severe bleeding after minor injuries, spontaneous bleeding and joint bleeding are rare; 6% to 20% are mild, patients with minor injuries or bleeding after surgery The time was prolonged, but there was no spontaneous bleeding or joint bleeding; 20% to 50% were subclinical types, and bleeding occurred only after severe trauma or surgery.

Hemophilia type B bleeding symptoms and severity classification are similar to hemophilia A. Factor activity of less than 2% is severe and rare; the vast majority of patients are mild. Therefore, the bleeding symptoms of this disease are mostly mild.

(Two) laboratory inspection

The disease is mainly an endogenous coagulopathy, so bleeding time, platelet count and morphology, PT, TT, and von Willebrand factor-associated antigen (vWF: Ag) are normal. APTT is prolonged, and thrombin production is poor. Identification of the two types can be used to correct the thromboplastin time. The : C and factor activities (: C) were measured to estimate their concentrations in plasma.

(Three) diagnosis

According to the typical clinical manifestations and laboratory APTT, thromboplastin test and correction test, and coagulation factor activity measurement, the diagnosis and differentiation of hemophilia A and B are not difficult. However, it needs to be distinguished from the lack of factor , which is an incomplete recessive inheritance of autosomes, which can be affected by both men and women, and can be transmitted by both parents. The clinical bleeding symptoms are mild. According to poor thromboplastin production, normal adsorption of plasma and normal serum can be corrected, and the reduction or disappearance of plasma factor activity can be identified. In addition, it needs to be distinguished from vascular hemophilia and the presence of anticoagulant substances (factors , inhibitors) in the circulation. The latter has the same bleeding symptoms as hemophilia, but has no family history, gender, age restriction, and abnormal blood clotting. Cannot be corrected by small amounts of normal plasma.

(D) Treatment

1. The bleeding principle of hemophilia supplemented with coagulation factors is to supplement the lack of coagulation factors to increase the plasma factor concentration to hemostatic levels. Generally available fresh plasma, severe bleeding must be surgery, or patients with heart failure should use anti-hemophilia globulin concentrate, cryoprecipitate or prothrombin complex concentrate (containing factors , X, , ).

2. DDAVP is a kind of synthetic anti-diuretic hormone. It has the effects of anti-diuresis and mobilization of storage factor in the body. It is mainly used in patients with mild hemophilia A. It is commonly used in clinical practice of 0.3 0.5 g / kg. Intravenous injection of 20-30 mL of saline can also be used for nasal drops at a high concentration of 1 g / kg, once every 12 hours.

3 Anti-fibrous solvents can protect the formed blood clots from dissolving, and can be used to stop bleeding in oral wounds and tooth extractions. Anti-fibrous solvents are often used in combination with complementary therapies. 6-aminocaproic acid 4-6g is usually used 4 times a day, and the total amount is 20 25g, 72 to 96 hours after tooth extraction. Can also be used p-aminotoluic acid (PAM BA) 100 ~ 200mg each time into the glucose solution intravenous bolus or drip. Tranexamic acid. Should pay attention to cause hematuria, the formation of small blood clots in the urethra can cause the danger of urinary tract obstruction.

4 Local hemostatic deep tissue hematoma and joint bleeding should be avoided, rest in bed, and place the affected limb in a comfortable position. Repeated bleeding should pay attention to placing the limb in a functional position, using ice packs or bandages for local compression and fixation. After the bleeding stops, the local hematoma disappears and can be appropriately moved.

Coagulopathy

Vascular hemophilia (von Willebrand disease, vWD) is a hereditary hemorrhagic disease. It is clinically characterized by a tendency to bleed from an early age, prolonged bleeding time, decreased platelet adhesion, and induced by ristomycin. Platelet agglutination is weakened or non-agglutinating. Von willebrand factor (vWF) deficiency or abnormal molecular structure in plasma.

Normal human plasma factor is a protein complex composed of low molecular weight factor : C and high molecular weight vWF. The basic defect of patients with this disease is that the synthesis of vWF is impaired, resulting in reduced platelet adhesion and no agglutination of platelets to ristomycin. More than half of the patients also have a decrease in C :. At present, vWF is thought to be produced by vascular endothelial cells, so it is speculated that the disease of this disease may be in endothelial cells. The disease is autosomal dominant, and individual subtypes are recessive. Both men and women can contract the disease, both parents can pass it, and both parents have no symptoms.

(1) The clinical manifestations are bleeding. Generally, skin and mucous membrane bleeding are common. There are epistaxis, gum bleeding, skin ecchymosis, and excessive menstruation. In severe cases, there may be gastrointestinal bleeding, hematuria, bleeding after surgery and postpartum Major bleeding. Joint and muscle bleeding are rare. Bleeding symptoms most often occur in infants and young children. A few patients do not show bleeding symptoms until adulthood. The degree of bleeding gradually decreases with age.

The most significant diagnosis of this disease is based on prolonged bleeding time, decreased platelet adhesion, reduced or non-agglutination of platelet aggregation induced by ristomycin, and normal response to other inducers. Decreased plasma factor VII: C and vWF: Ag concentrations or abnormal vWF structure. Those with bleeding symptoms and family history can help confirm the diagnosis. The disease can be roughly classified into three types based on genetic methods, clinical manifestations, and laboratory tests: Type I: The most common type is autosomal dominant inheritance, which is mainly manifested by a decrease in the amount of vWF and a basically normal vWF multimer structure; Type: usually also autosomal dominant heredity, vWF multimers have structural and functional abnormalities, this type can be divided into subtypes such as IIA, IIB, and IIC; type III is heavy, autosomal recessive, vWF antigen of patients And the activity is extremely low or absent. The disease must be distinguished from hemophilia A (factor : C is reduced and vWF: Ag is normal), platelet weakness (platelet membrane glycoprotein b, a deficiency, and decreased platelet-induced aggregation rate).

(2) Treatment of mild patients can take local hemostatic measures and / or DDAVP treatment, which can promote the release of factor into the blood circulation and increase the plasma factor concentration in normal or light patients. Severe bleeding should be supplemented with factor , fresh plasma, or whole blood; cold precipitation and new vWF preparations can be used when available, the latter is rich in vWF and can be used for all hereditary vWD patients. Repeated menstruation can also take oral contraceptives to suppress menstruation. The lack of vWF multimers in factor VIII concentrated preparations is difficult to correct the bleeding of this disease, so it is not preferred. This disease prohibits drugs such as aspirin, pansentin, buta pine and low molecular dextran that affect platelet function.

K Vitamin K deficiency with coagulopathy

Vitamin K plays an important role in the coagulation process. When it is lacking, it can cause the deficiency of vitamin K-dependent clotting factors (prothrombin, factors , , and X). These factors require the participation of vitamin K, which is synthesized in the liver and released through the cell membrane. To extracellular. Severe deficiency often occurs with spontaneous bleeding.

Vitamin K can be divided into K1 (natural product, derived from green leafy vegetables), K2 (synthesized by bacteria parasitic in the small intestine or colon), and K3 (synthesized). The human body needs about 1 g / kg of vitamin K per day, and infants only need 1 g per day. The absorption of vitamin K in the intestinal tract requires the help of bile salts. The absorbed vitamin K is converted into activated epoxide (epoxidized chlorophyll) by the action of hepatocyte microsomal epoxidase, and then acts by microsomal reductase. The reduction is reduced to vitamin K; this redox process helps the carboxylase in the microparticles to convert glutamic acid in the precursor of vitamin K-dependent factors to ? Carboxyglutamic acid, and promotes the production of vitamin K-dependent coagulation factors. Therefore, vitamin K deficiency will affect the synthesis of vitamin K-dependent factors.

There are three main reasons for vitamin K deficiency: insufficient food intake; malabsorption caused by bile salt deficiency is seen in completely obstructive jaundice, drainage or fistulas after bile duct surgery and long-term oral antibiotics inhibit the intestinal bacterial population; oral and Vitamin K has antagonizing anticoagulants such as: Coumarins can accumulate epoxidized chlorophyll quinones and cannot be reduced to vitamin K. Or long-term oral antibiotics can inhibit the intestinal bacterial population and reduce vitamin K synthesis.

Clinical manifestations are skin petechiae, petechiae, and mucosal bleeding, which are generally mild. In addition, trauma, bleeding after surgery, hematuria, menstruation, and gastrointestinal bleeding often occur. No deep tissue bleeding and joint bleeding were seen.

The laboratory is characterized by prolonged PT, prolonged APTT, and normal TT. The determination of factor , , , X activity is of great help in the diagnosis.

Treatment should first remove the various causes of vitamin K deficiency or treat the primary disease, and actively supplement vitamin K. However, oral absorption of vitamin K25 to 100mg is poor. Intramuscular injection can cause severe vitamin K deficiency and hematoma, so those who have bleeding tendency can slowly inject vitamin K10 ~ 15mg. If the cause can not be removed, it may need to be injected once a month.

Coagulation disorders caused by severe liver disease

The liver plays an important role in the synthesis and metabolism of coagulation factors. Except for tissue factors and factor IV (Ca2 +), almost all coagulation factors are synthesized in the liver. Known are fibrinogen, prothrombin, factors X, X, , V, and some factor , etc .; the synthesis sites of factors , , and have not been determined. Most blood coagulation factors have severe plasma levels in severe liver disease decline. At the same time, the liver can also synthesize inhibitors of plasminogen and plasminogen activator. Production of anticoagulant factors (antithrombin III, protein C, protein S). The liver still clears plasmin, activated coagulation factors, and fibrin (pro) degradation products. When liver diseases, especially the synthesis of vitamin K-dependent coagulation factors and fibrinogen are reduced, dysfunction is eliminated, antithrombin III and plasminogen synthesis are reduced, and DIC and liver disease can occur in severe liver disease and liver failure. Primary hyperfibrinolysis; portal hypertension, congestive splenomegaly, thrombocytopenia, etc. can cause abnormal blood clotting.

Clinical manifestations In addition to the original symptoms of liver disease, skin and mucous membrane bleeding such as epistaxis, bleeding gums, petechiae, excessive menstruation, severe cases may have vomiting and melena. PT, APTT, and TT can be prolonged in the laboratory, and thrombocytopenia may be present. Progressive thrombocytopenia, prolonged PT, decreased fibrinogen, and a positive 3P test all suggest concurrent DIC and require further examination.

Treatment should be based on the treatment of liver disease. Hemorrhage is obvious, and fresh plasma, whole blood or fresh frozen plasma and prothrombin complex preparation can be transfused when PT is prolonged to supplement coagulation factors. Heparinoids can be treated with protamine sulfate when there is an increase in heparin. When liver disease is combined with DIC, DIC should be used for treatment, but the application of heparin needs to be careful.