intra-articular hemorrhage
Introduction
Introduction Joint bleeding is common in hemophiliacs, with the most common bleeding being the knee, elbow, and ankle joints. After the blood is deposited in the joint cavity of the patient, the joint activity is limited and the function is temporarily lost. For example, the patient often cannot stand and walk normally after the knee joint bleeding. The blood deposited in the joint cavity often takes several weeks to be gradually absorbed, and gradually restores function. However, if the joint repeatedly bleeds, it can cause synovitis and arthritis, causing joint deformity and making the function of the joint difficult to return to normal. Therefore, many people with hemophilia have different degrees of disability.
Cause
Cause
Joint bleeding is common in hemophiliacs, with the most common bleeding being the knee, elbow, and ankle joints. After the blood is deposited in the joint cavity of the patient, the joint activity is limited and the function is temporarily lost. For example, the patient often cannot stand and walk normally after the knee joint bleeding. The blood deposited in the joint cavity often takes several weeks to be gradually absorbed, and gradually restores function. However, if the joint repeatedly bleeds, it can cause synovitis and arthritis, causing joint deformity and making the function of the joint difficult to return to normal. Therefore, many people with hemophilia have different degrees of disability.
Examine
an examination
Related inspection
Bone and joint soft tissue CT examination activated partial thromboplastin time (APTT) prothrombin time (PT) arthroscopy plasma thrombin detection
1, bleeding characteristics
Spontaneous or minor trauma, see oozing, even for several days, mostly ecchymosis, hematoma; knee, ankle, elbow, wrist and other joints are prone to bleeding, repeated bleeding can cause joint deformity, oral and nasal mucosal bleeding is also more common.
2, laboratory inspection
1 coagulation examination showed prolonged clotting time (light normal), poor prothrombin consumption (about 70% of patients). 2 Coagulation factor was abnormally measured.
Diagnostic guidance for hemophilia
Male patients with repeated joint hemorrhage or deep hematoma formation, plasma FVIII: C or FIX: C less than % bleeding family history of bleeding can diagnose hemophilia (A or B), if FVIII: C levels are reduced but family history is not Typical (or only ~ male bleeding patients) vascular pseudohemophilia can not be excluded.
Laboratory features: hemophilia patients with normal blood vessel wall and platelet function, so bleeding time (BT) is normal, patients have normal fibrinogen and factor II, VII, V activity, so prothrombin time (PT) is normal, but there are Functional coagulation factor VIII activity or IX deficiency, abnormality of endogenous coagulation system test, diagnosis must be based on in vitro coagulation activity screening test, partial thromboplastin time (APTT or KPTT) or thromboplastin generation time (biggs TGT).
If the patient's BT, PT, TT (thrombin time) are normal and the APTT is prolonged, it is necessary to further determine plasma FVIII: C or FIX levels, which may help to classify by severity. FVIII: C is present in fresh plasma adsorbed by normal and barium sulfate or aluminum hydroxide, but not in serum; FIX is present in normal serum and not in adsorbed plasma, so if APTT (KPTT) or Biggs TGT It can be corrected by normal adsorption plasma without being corrected by normal serum, then it can be qualitatively diagnosed as hemophilia A; if the abnormality is corrected by normal serum and not corrected by adsorbed plasma, it can be qualitatively diagnosed as hemophilia. B; the clinical symptoms of patients with von Willebrand's disease are similar to those of hemophilia, but both sexes may be ill, the patient's plasma lacks von Willebrand's factor (VWF), one can bind to the platelet membrane and participate Platelet adhesion and platelet-platelet interaction protein VWF and FVIII:C carrier protein binds VIII:C non-covalently in plasma to stabilize FVIII:C, so VWD patients due to VWF (VIII: The decrease or lack of C carrying molecules shortens the FVIII:C half-life, the patient's FVIII/VWF level decreases, and the FVIII:C level decreases, and the bleeding time prolongs the platelet's aggregation reaction with ristomycin, so Hemophilia is different.
In the second trimester, a special small-pore fetal lens is used to obtain pure fetal blood (no amniotic fluid or maternal blood) for FVIII: C and FVIII: CAg determination can determine whether the fetus is a hemophilia patient, hemophilia patient FVIIIC: Ag often decreases in consistency with FVIII:C activity, and a few can be normal. Therefore, if the fetal blood FVIIIC:Ag value is significantly lower than %, hemophilia A can be diagnosed, while normal can exclude moderate to severe hemophilia. In recent years, the application of genetic diagnosis methods has a low risk and a high correct rate, but it has high technical requirements and has not yet been promoted.
Diagnosis
Differential diagnosis
Hemophilia should be identified as follows
1 Identification between hemophilia A and hemophilia B
Hemophilia A (HA): is a type of X-linked coagulation factor VIII and a recessive hereditary hemorrhagic disease caused by abnormal molecular structure. The clinical features are "spontaneous" joint hemorrhage and deep tissue bleeding. .
Hemophilia B (HB): is a hereditary disease with a genetic pattern and hemorrhagic behavior similar to that of hemophilia A. Its pathogenesis is the lack of factor IX.
2 Identification of von Willebrand disease
Vascular hemophilia: Also known as VonWillebrand syndrome (referred to as VWD). The patient's plasma has a lack of VonWillebrand factor (VWF) or abnormal molecular structure. The typical case performance is: 1 prolonged bleeding time. 2 platelets have reduced adhesion to glass beads and have reduced or no aggregation of ristocetin aggregation. 3 Plasma factor VIII related antigen (VIIIR: Ag) and clotting activity (VIII: C) decreased or VWF activity (VIIIR: VWF) decreased. VWD is a relatively common hereditary hemorrhagic disease, which can be criticized by both men and women. Most patients are autosomal dominant, a few are autosomal recessive, and the VWF gene is located on chromosome 12.
3 Identification of diseases associated with other coagulation factors.
