Acquired von Willebrand disease (aVWD) in a lymphoplasmocitary/ Waldenström Macroglobulinemia lymphoma
Case report
Abstract
Acquired Von Willebrand disease is an unusual situation arising within the context of self-immune diseases, lymphoproliferative and mieloproliferative disorders. The study presents the clinical case of a patient carrier of a lymphoplasmocitary lymphoma with Waldenström's disease which presented with a hemorrhagic syndrome and alterations of the blood crasis in the intrinsic way. Monoclonal gammopathies such as Waldenström's macroglobulinemia usually appear with varied IgM dosifications, being it > 11 g/dl in the case described. Clinical symptoms may be very proteiform, affecting several systems, and in this case it presented complications resulting from tumor adsorption: acquired Von Willebrand disease. Chemotherapy with thalidomide, cyclophosphamide and dexamethasone was indicated for the base disease and there was favourable evolution, the hemorrhagic syndrome remitted and there was a tendency to crasis normalization, the same as globular and platelets values ongoing normalization after six series of treatment. Other therapeutic measures aiming to reverse coagulopathy, such as plasmapheresis, are short-acting, and they are not exempt from risks, and they are considered as options upon obvious acute hyperviscosity with a risk of life. Multidisciplinary patient assessment enabled the best diagnosis and therapeutic approach.
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