The Von Willibrand's Factor (vWF) is an glycoprotien found primarily in the alpha-granules of the platlets, as well as in collegen (ie. connective tissues) Its primary function is binding to other proteins, particularly Factor VIII and it is important in platelet adhesion to wound sites. In other words it is important in blood clotting.
Low-levels of vWF can be associated with bleeding disorders, symptoms include mainly mucousal bleeding (gums, nasal membranes, or heavy menstural periods) The disorder can be very mild (so it is never even diagnosed) or more severe leading to risks of bleeding with surgical procedures or anemia due to heavy mensturation (rarely is it life-threatening). This disorder is hereditary.
Antibodies to vWF are found in thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). This is called aquired Von Willibrands Disease.
I have had these tests done, I have hereditary vWD. As well as my daughter. This has however nothing to do with my MCTD. And is not auto-immune linked.
If your doctor is ordering these tests, he may suspect that you have an aquired form of vWD. Have you had bleeding problems? or clotting problems (Thrombocytopenia)?
Normally a genetic vWD would be evaluated by a hemotologist.
Thanks for your info, very helpful
The doctor didn’t say anything about bleeding problems, but she didn’t explain a lot at last app’t – I was in rough shape and wouldn’t have retained it anyway, she was giving me written instructions for basic stuff at that point. I know that her note to my GP said that she was concerned about CNS involvement from my Lupus and also she mentioned suspecting vasculitis (she referred to a rash on my forearms and neck). I don’t know if my labs showed anything in the way of platelets, etc, I haven’t been informed and my GP just says it’s too early to hear back from my Rheumy about anything (although she has faxed her report twice ). My mom, who has Lupus, has thrombocytopenia quite severely at times, and I know she has mentioned some purpura thing but I can’t for the life of me recall what it was, will ask her.
Lately, it seems I can’t keep things straight sometimes even to do tasks around the house, I get confused, and my memory is shot. I have a medical background and used to know all of this stuff but lately I can't even remember whether I let the dog back in the house (not good when it's -35C....brrr)
I can understand the frustration of not being able to remeber simple things. I forget everything I am supposed to do if I don't write it down (then I usually forget where I put the list!). I too have a medical background (Pathologist) luckily despite CNS involvement I haven't forgotten everything!
I assume your mother has ITP (immune thrombrocytopenic pupura)-is the condition of having a low platelet count (thrombocytopenia) of no known cause (idiopathic). Most cases appear to be related to antibodies against platelets, or platelet factors. Most often the antibodies are against platelet glycoprotiens of the IgG type.
Treatment options for ITP are Prednisone, IVIG (intervenous immunogloubins), immunsuppressives, and sometimes Splenectomy (removal of the spleen, as this is where most of the platelets are destroyed).
Since ITP is generally a diagnosis of exclusion, test will be run first to look for other causes of bleeding and/or low platelet counts. Secondary causes could be leukemia, medications (e.g. quinine, heparin), lupus, cirrhosis, antiphospholipid syndrome, von Willebrand factor deficiency and others.
Depending on your platelet counts, it could be that your doctor wants to check out these other possible causes for ITP. But here we are really speculating. When you have the results, and can talk with the doctor I am sure she will explain what she was looking for.