Successful desensitization in a patient with Von Willebrand´s disease and anaphylaxis to factor VIII/VW

نویسندگان

  • Maristela Olival
  • Mara Felix
  • Monica Soares
  • Marilia Renni
  • Silmara Montalvao
  • Luis Ensina
  • Mariana Castells
چکیده

Background Von Willebrand ́s disease (VWD) is the most common congenital disorder of hemostasis, characterized by deficient or defective von Willebrand factor. Patients are treated by intravenous replacement of factor VIII/VW (FVIII/ VW) when needed, for prophylaxis before surgical procedures. Anaphylactic reactions to FVIII/VW are rare and desmopressin (DDAVP) can be used as an alternative.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Role of carbohydrate in multimeric structure of factor VIII/von Willebrand factor protein.

The carbohydrate moiety of the factor VIII/von Willebrand (vW) factor protein is important in the expression of vW factor activity and the intravascular survival of the protein. Studies of normal human factor VIII/vW factor protein indicate that there is a requirement of a full complement of penultimate galactose for the maintenance of a normal multimeric structure. Release of penultimate galac...

متن کامل

Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding

A 91-year-old woman affected with acquired Von Willebrand (VW) syndrome and intestinal angiodysplasias presented with severe gastrointestinal bleeding (hemoglobin 5 g/dl). Despite replacement therapy with VW factor/factor VIII concentrate qid, bleeding did not stop (eleven packed red blood cell units were transfused over three days). High circulating levels of anti-VW factor immunoglobulin M we...

متن کامل

Selective absence of large forms of factor VIII/von Willebrand factor in acquired von Willebrand's syndrome. Response to transfusion.

A previously healthy elderly man with mucocutaneous bleeding was found to have a benign monoclonal IgG gammapathy associated with criteria for severe von Willebrand disease (Factor VIII procoagulant activity, Factor-VIII-related antigen, and ristocetin cofactor activity, less than 10% of normal). Associated qualitative abnormalities of factor VIII/von Willebrand factor were demonstrated by radi...

متن کامل

Selective Absence of Large Forms of Factor Vill/von Willebrand Factor in Acquired von Willebrand’s Syndrome. Response to Transfusion

A previously healthy elderly man with mucocutaneous bleeding was found to have a benign monoclonal lgG gammapathy associated with criteria for severe von Willebrand disease (Factor VIII procoagulant activity, Factor-VIII-related antigen, and ristocetin cofactor activity, <10% of normal). Associated qualitative abnormalities of factor VIII/von Willebrand factor were demonstrated by radiocrossed ...

متن کامل

Effect of multimeric structure of the factor VIII/von Willebrand factor protein on binding to platelets.

The characteristics of the intact factor VIII/von Willebrand factor protein binding to human platelets was compared to 2-mercaptoethanol-treated factor VIII/von Willebrand factor protein and to fractions of plasma factor VIII/von Willebrand factor protein that elute after the void volume. These studies indicate that the factor VIII/von Willebrand factor protein larger size oligomers bind prefer...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 4  شماره 

صفحات  -

تاریخ انتشار 2014