Vascular pseudohemophilia (VWD) is second only tohemophiliaMost common heritability ofHemorrhagic disease。It is characterized by hemorrhagic tendency since childhood, prolonged bleeding time and decreased factor VIII content.VWD is caused by the lack of large molecular weight part of coagulation factor Ⅷ, namely Ⅷ R.Ⅷ R is inherited by autosome, and Ⅷ: C is inherited by X chromosome.Typical VWD not only reduces the activity of factor Ⅷ related antigen (Ⅷ R: Ag), i.e. antigen part of Ⅷ R, but also reduces or lacks the factor Ⅷ restorin cofactor (Ⅷ R: RCOF), and also reduces Ⅷ: C, but to a lesser extenthemophiliaA is serious.Ⅷ R: Ag and Ⅷ R: RCOF are important factors for the adhesion of platelets to each other and between platelets and vascular endothelium, and are cofactors for inducing platelets to aggregate Restolase. Due to the lack of such factors in this disease, the adhesion and aggregation function of platelets is hindered, and the bleeding time is prolonged.
Vascular pseudohemophiliaThe inheritance mode of the disease is autosomal dominant inheritance, and the individual subtype is recessive inheritance.Both men and women can get sick, both parents can pass on, and some patients' parents can have no bleeding symptoms.The disease tends to bleed in childhood, and a few patients show clinical symptoms after adulthood.The bleeding symptoms and typicalhemophiliaSimilar, but slightly less serious.The disease may decrease with age.
clinical manifestation
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1. Family history: It conforms to the rule of chromosome dominant inheritance, that is, both men and women can get sick, and both parents can inherit.
2. Bleeding tendency: mucosal, subcutaneous bleeding orMenorrhagiaThere is or is no serious bleeding history after the operation. A few joints and muscles bleed, but generally there is no joint deformity.
Diagnostic differentiation
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Diagnostic basis
（1） With or without family history, those with family history conform to the autosomal dominant or recessive inheritance law.
（2） Clinical bleeding of mucous membrane, skin and internal organs orMenorrhagiaHistory, trauma, surgery with or without history of abnormal bleeding, a few patients can be related to the phenomenon of bleeding in the segment, muscle or other parts.
（3） Laboratory tests to aid diagnosis
1. Platelet morphology and count are normal.
2. Prolonged bleeding time or positive aspirin tolerance test.
3. Platelet adhesion test is prolonged or normal.
4. The activated partial thromboplastin time is prolonged or normal.
5. The VWF factor antigen (VWF: Ag) is reduced or normal.
6. Factor Ⅷ coagulation activity (Ⅷ: C) is reduced or normal.
7. Platelet function deficiency diseases must be excluded.
Disease treatment
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Therapeutic principles
1. Prevention and treatment.
2. Supplement coagulation factors.
3. Symptomatic support therapy.
Principles of medication
Anti platelet aggregation drugs are forbidden in patients with vascular pseudohemophilia.Generally, treatment with "A" and "B" drugs can improve bleeding symptoms, and blood products need to be transfused before and after surgery.
