Dyscrasies plasmocytaires : formes rares et atypiques

Abstract

Plasma cell dyscrasias, other than classical symptomatic multiple myeloma, can take very various clinical presentations. AL amyloidosis, monoclonal immunoglobulin deposition disease or monoclonal gammopathy of clinical significance look like indolent diseases : a small clone of plasma cells produces low amounts of monoclonal immunoglobulins that deeply damage some organs, especially kidney, skin or heart. On the other hand, extramedullary myeloma disease or plasma cell leukemia are highly aggressive diseases related to the spread of tumor cells outside of the bone marrow. Prognostic of these presentations remains appalling, even with the introduction of new promising treatments. For these unusual forms, the best strategy remains unclear but often relies to the eradication of clonal plasma cells, using classical chemotherapy (alkylating agents), but also proteasome inhibitors, immunomodulatory drugs, and more recently monoclonal antibodies.