Hematology Disease Topics & Pathways:
multiple myeloma, Diseases, Plasma Cell Disorders, Lymphoid Malignancies
Rare at diagnosis, but more frequent at the time of relapse, are less typical disease presentations such as plasmacell leukemia, extramedullary disease and central nervous system (CNS) involvement. These clinical features, mostly due to very aggressive disease, are also associated with poor prognosis, and require prompt, proper and often aggressive management, with a careful look at preventing and managing adverse events to improve survival. This session will focus on the complexities of providing optimal management to patients with “difficult to treat MM”, presenting with renal failure, plasmacell leukemia, extramedullary and CNS disease.
Dr. Francesca Gay will review the main pathogenetic mechanisms of renal damage in MM and treatment guidelines. The main focus will be on patients with severe renal insufficiency, including patients on dialysis. Specific and effective treatment strategies to achieve recovery of renal function will be addressed. These will include supportive care and anti-MM therapy. Evidence on the use of different novel agents (proteasome inhibitors, immunomodulatory agents, monoclonal antibodies) in patients with renal insufficiency will be summarized. Furthermore, how to optimally and safely administer each of them will also be addressed.
Dr. Laura Rosinol will review the incidence, types, prognosis and treatment recommendations of the extramedullary involvement in MM. The prognosis of the extramedullary disease at diagnosis and at relapse will be analyzed, as well as the efficacy of novel drugs. The presentation and treatment approach of CNS involvement, a condition with extremely poor prognosis, will be reviewed. Plasma cell leukemia is a rare entity with an aggressive course. The diagnostic criteria as well as the therapeutic algorithm will be discussed.