Enfermedad de Von Willebrand adquirida en un linfoma linfoplasmocitario/Macroglobulinemia de Waldenström: Reporte de caso

Isabel Moro; Carolina Oliver; Mariana Stevenazzi; Cecilia Guillermo; Silvia Pierri; Jorge Decaro

Authors

Isabel Moro Universidad de la República, Facultad de Medicina, Cátedra de Hematología. Asistente
Carolina Oliver Universidad de la República, Facultad de Medicina, Cátedra de Hematología. Ex Residente
Mariana Stevenazzi Universidad de la República, Facultad de Medicina, Clínica Médica B. Profesora Adjunta
Cecilia Guillermo Universidad de la República, Facultad de Medicina, Laboratorio Clínico. Profesora Adjunta
Silvia Pierri Universidad de la República, Facultad de Medicina, Cátedra de Hematología. Ex Profesora Adjunta
Jorge Decaro Universidad de la República, Facultad de Medicina, Cátedra de Medicina Transfusional. Profesor

Keywords:

VON WILLEBRAND DISEASES, WALDENSTROM MACROGLOBULINEMIA

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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