Differential Diagnosis of von Willebrand disease
A differential diagnosis for von Willebrand disease is performed with hemophilia, thrombocytopathy / thrombocytopenia and hereditary hypoconvertinemia. Hemophilia is a hereditary disease of the hemostatic system, characterized by a decrease or impaired synthesis of coagulation factors VIII or IX (Flood, Schlauderaff and Haberichter, 2015). The disease is inherited in an autosomal recessive manner, linked to the X chromosome. This determines that hemophilia has males and women who transmit the pathological gene. A characteristic manifestation of hemorrhagic syndrome is hematoma type of bleeding and the development of hemarthrosis. In the coagulogram, an isolated extension of the APTT, a decrease in the activity of factor VIII below 50%, and the absence of a disturbance on the part of the VWF, VWF: RCo antigen content, von Willebrand factor multimers are determined.
Thrombocytopenia is a disease characterized by petechial-bruising type of bleeding. In blood tests, a decrease in the number of platelets, a change in the morphology of platelets is recorded in different degrees of severity (Longing, Christophe and Denis, 2015). At the same time, in the coagulogram, the prothrombin time, activated partial thromboplastin time, fibrinogen content is not changed, the content of coagulation factors is within normal limits. The duration of bleeding is normal or increased. The diagnosis is established on the basis of electron microscopy of platelets, platelet aggregation studies with different inducers.
The disease is manifested by bleeding tendency (Torisu, Torisu and Lee, 2013). Most children have bleeding tendency in infants and young children, and the severity of bleeding varies (Laffan et al 2014). The tendency to hemorrhage can be reduced with age.
Transfusion therapy, including whole blood and plasma infusion to supplement the lack of factors, especially anti-hemophilia globulin lacking in hemophilia A is one of the most effective method (Laffan et al 2014). In the case of severe bleeding, the above-mentioned whole blood and plasma injection cannot increase the amount of AHG to the amount of hemostasis required due to excessive blood circulation.
Torisu, T., Torisu, K. and Lee, I. H. (2013). Autophagy regulates endothelial cell processing, maturation and secretion of von Willebrand factor. Nat Med. 9(10):1281–1287.
Longing, P. J., Christophe, O. D. and Denis, C. V. (2015). von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 125(13):2019–2028.
Flood, V.H., Schlauderaff, A. C. and Haberichter, S. L. (2015). Zimmerman Program Investigators . Crucial role for the VWF A1 domain in binding to type IV collagen. Blood. 125(14):2297–2304.
Laffan M. A. et al (2014). The diagnosis and management of von Willebrand disease: A United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov; 167(4): 453–465.
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