Menstruation is a stage that every woman needs to go through. During the menstrual period, the female's uterus is open. During this period, the waste toxins in the uterus will be discharged from the body with menstrual blood. The normal menstrual period is about 7-5 days. If it is too long or too short, it is a problem. If you keep having menstruation, you may have a uterine disease, which can easily lead to bleeding and requires treatment in the hospital. What should I do if I keep having my period? If you have continuous menstruation, you may have a coagulation disorder. You need to check your coagulation function and blood routine to rule out blood system diseases. If there is no abnormality in the blood system, you need to do a uterine B-ultrasound to see if there is any endometrial lesion to rule out endometrial tumors. Clinical classification It is divided into two categories: hereditary and acquired. 1. Hereditary coagulation disorders are generally caused by a single coagulation factor deficiency, which often causes bleeding symptoms in infancy and often has a family history. 2. Acquired coagulation dysfunction is relatively common. Patients often have multiple coagulation factor deficiencies. It mostly occurs in adults. Clinically, in addition to bleeding, there are also symptoms and signs of the primary disease. Classification Description hemophilia Hemophilia is the most common hereditary coagulation factor deficiency, which can be divided into two types: hemophilia A (factor VIII procoagulant component deficiency) and hemophilia B (factor IX deficiency). The biosynthetic genes of factor VIII:C and factor IX are both located on the X chromosome, so they are called X-linked diseases. Both are X-linked recessive inheritance, occurring in males and transmitted to females. Although female carriers have varying degrees of reduced factor VIII:C or factor IX activity, they generally have no bleeding symptoms. About 1/3 of patients have no family history. This may be because there are few males in the family or the disease is inherited from the previous generation and is overlooked, or it may be caused by gene mutation. 1. Clinical manifestations The main symptom is bleeding, characterized by bleeding in soft tissues, muscles, and weight-bearing joints. Bleeding tendency usually occurs in early childhood, and mild cases may not be diagnosed until adolescence or even adulthood. The earlier the bleeding symptoms appear, the more serious the condition. Patients may present with severe bleeding after minor trauma or surgery, often with bleeding that does not stop during tooth extraction or minor surgery. A small number of patients experience this as the first symptom. Bleeding can continue for hours or even weeks. The degree of bleeding is related to the plasma factor activity (concentration). Although the activity of factor VII or IX required for normal hemostasis is 25%, the factor activity in symptomatic patients is often less than 5%. Clinically, hemophilia is divided into severe, moderate, mild and subclinical types based on factor activity. The most common sites of bleeding are the vulnerable parts of the limbs. Deep tissue hematomas may occur. Large hematomas may compress nearby nerves such as the femoral nerve, median nerve, and ulnar nerve, causing pain and paralysis symptoms; compression of blood vessels may cause jaundice. Bleeding from the soft tissues of the neck and throat may cause suffocation due to airway obstruction. Retroperitoneal and mesenteric bleeding may cause abdominal pain. Severe cases may experience epistaxis, gingival bleeding, gastrointestinal bleeding, and hematuria. Excessive bleeding may cause anemia. Recurrent bleeding into the joint cavity occurs in critically ill patients, often after minor injuries, and may also occur spontaneously. There may be local swelling, pain, tenderness, and acute symptoms lasting 3 to 5 days. After the bleeding stops, the accumulated blood will be gradually absorbed after about a few weeks and no trace will be left. If it is not absorbed over time, it can cause synovitis, and repeated bleeding can cause joint stiffness, and finally lead to permanent joint damage, osteoporosis, limited joint movement, deformation, and nearby muscle atrophy, resulting in disability. The most commonly affected joints are the ankles in infants and young children and the knees in children and adults. ◇The relationship between the deficiency of coagulation factors A and B and the degree of bleeding in hemophilia A and B Hemophilia A and B may develop in the neonatal period but most develop by age 2 years. The severity of the bleeding in the former is related to the activity of VIII:C in their plasma: those with an activity of 0-1% are severe, and the patients have spontaneous bleeding, repeated joint bleeding or deep tissue (muscle, visceral) bleeding since childhood, and often lead to joint deformities; 2%-5% are moderate, and the patients have severe bleeding after minor injuries, and spontaneous bleeding and joint bleeding are rare; 6%-20% are mild, and the patients have prolonged bleeding time after minor injuries or surgery, but no spontaneous bleeding or joint bleeding; 20%-50% are subclinical types, and there is only oozing of blood after severe trauma or surgery. The bleeding symptoms and severity classification of hemophilia B are similar to those of hemophilia A. Those with factor IX activity less than 2% are severe and very rare; the vast majority of patients have mild symptoms. Therefore, the bleeding symptoms of this disease are mostly mild. 2. Laboratory examination This disease is mainly an intrinsic coagulation disorder, so the bleeding time, platelet count and morphology, PT, TT, and von Willebrand factor-related antigen (vWF: Ag) are all normal. APTT is prolonged and thromboplastin generation is poor. A thromboplastin generation time correction test can be performed to differentiate between the two types. The activity of factor VIII:C and factor IX (IX:C) was measured to estimate their concentrations in plasma. (III) Diagnosis Based on the typical clinical manifestations and laboratory tests such as APTT, thromboplastin generation test and correction test, and coagulation factor activity assay, the diagnosis and differentiation of hemophilia A and B are not difficult. However, it needs to be differentiated from factor XI deficiency, which is an autosomal incomplete recessive inheritance that can affect both men and women, can be passed on from both parents, and has milder clinical bleeding symptoms. It can be identified based on poor thromboplastin generation, which can be corrected by normal adsorbed plasma and normal serum, as well as decreased or absent plasma factor XI activity. In addition, it is necessary to differentiate it from von Willebrand disease and the presence of anticoagulants in the circulation (factor VII and IX inhibitors). The latter has the same bleeding symptoms as hemophilia, but has no family history and no gender or age restrictions, and the coagulation abnormalities cannot be corrected by a small amount of normal plasma. |
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