von Willebrand Factor Deficiency
Acquired vWD is a rare disease, with up to 4.5% of patients with a clinical and laboratory diagnosis of vWD may have the acquired form. The pathogenesis of this disease, inhibition of von Willebrand factor (vWF) as part of a generalized autoimmune reaction or development of specific anti-vWF antibodies. This can be seen in numerous hematological disease, such as NHL, antiphospholipid syndrome, MGUS, MM, Waldenstrom’s macroglobulinemia, CLL, angiodysplasia, polycythemia rubra vera. The specific inhibitor in many of the above mentioned disorders was found to be located in purified immunoglobulin (Ig)G fractions of the patients’ sera. In contrast, in others, the vWF inhibition was mediated by the disease-related monoclonal proteins.
References:
1) Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis, and treatment. Am J Med. 1997;103:536-540.