coagulation disorders
Introduction
Introduction Coagulation dysfunction 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.
Cause
Cause
1. Hereditary coagulopathy is generally a single clotting factor deficiency, mostly bleeding symptoms in infants and young children, often family history.
2. Acquired coagulopathy is more common, patients often have a variety of clotting factors, mostly in adulthood, clinically in addition to bleeding with the symptoms and signs of the primary disease.
Hemophilia is the most common group of hereditary coagulation factor deficiency, which can be divided into hemophilia A (factor VIII procoagulant component, VIII deficiency) and hemophilia B (factor IX deficiency). Factor VIII: C and Factor IX biosynthetic genes are located on the X chromosome, so it is called X? chain disease, both of which are X-linked with recessive inheritance, male onset, female transmission. Although female carriers have varying degrees of factor VIII: C or factor IX activity is reduced, but generally no bleeding symptoms. About one-third of patients have no family history, which may be neglected by males in the family or inherited from each other, or may be caused by genetic mutations.
Examine
an examination
Related inspection
Platelet activating factor simple thrombin generation test platelet factor III effectiveness test simple thrombin generation test plasma kallikrein assay
Laboratory inspection
The disease is mainly endogenous pathway coagulopathy, so bleeding time, platelet count and morphology, PT, TT, von Willebrand factor-related antigen (vWF: Ag) are normal. Prolonged APTT, poor production of thromboplastin. Identification of two types can be used to correct the thromboplastin generation time. VIII:C and Factor IX activity (IX: C) were determined to estimate its concentration in plasma.
diagnosis
According to the typical clinical manifestations and laboratory APTT, thromboplastin production test and correction test, blood coagulation factor activity determination, diarrhea A, B diagnosis and identification is not difficult. However, it needs to be differentiated from the lack of factor XI. The latter is an autosomal incomplete recessive inheritance. Both men and women can get sick, parents can pass, and clinical bleeding is mild. According to the poor production of thromboplastin, normal adsorption of plasma and normal serum can be corrected, and the reduction or disappearance of plasma factor XI activity can be identified. In addition, it needs to be differentiated from von Willebrand disease and circulating anticoagulant substances (factors VII and IX inhibitors). The latter has the same bleeding symptoms as hemophilia, but no family history and gender, age restriction and coagulation abnormalities. Can not be corrected by a small amount of normal plasma.
Diagnosis
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. 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 microthrombotic, 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.
3. Coagulation dysfunction: 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.
