How I treat extramedullary myeloma

Abstract

Extramedullary myeloma (EMM) is defined by the presence of plasma cells (PCs) outside the bone marrow in a patient with multiple myeloma (MM). Using sensitive imaging techniques including magnetic resonance imaging and positron emission tomography/computed tomography, EMM may be found in up to 30% of MM patients across the overall disease course. The molecular mechanisms underlying the hematogenous spread of PCs outside the bone marrow are only partially known and involve hypoxia and an altered expression of adhesion molecules. Extramedullary disease is associated with adverse prognostic factors (ie, high lactate dehydrogenase level, 17p deletion, and high-risk gene expression profile). The prognosis of EMM is poor, and the median overall survival of patients who experience an extramedullary relapse is <6 months. The adverse prognosis is less pronounced in patients with bone-related plasmacytomas than in those with hematogenous EMM. EMM patients should be considered as having high-risk myeloma and treated accordingly. However, EMM clinical situations are extraordinarily heterogeneous, and their management is particularly challenging. In the present review, a case-and-comment format is used to describe our approach to the management of EMM.

Successful treatment of central nervous system myeloma manifesting as cauda equina nodules with intrathecal chemotherapy, lenalidomide and dexamethasone

“…Extramedullary involvement with myeloma is seen in less than 5% of patients, while CNS involvement is extremely rare, accounting for approximately 1% of patients 2. Intraparenchymal lesions, solitary cerebral plasmacytomas and leptomeningeal disease have been documented previously 3.…”

Pomalidomide in the Management of Relapsed Multiple Myeloma

“…Studies in rodent showed that immunomodulatory compounds (including pomalidomide) cross the blood-brain barrier. Despite a lack of clinical data, immunomodulatory drugs, including pomalidomide, should be considered for the treatment of patients with CNS involvement [45,47].…”

“…Extramedullary disease (EMD), defined by the presence of plasma cells outside the bone marrow, is an independent adverse prognostic factor and such patients should be considered as having high-risk myeloma [45]. Short et al…”

Extramedullary Involvement of Lymph Nodes in Multiple Myeloma

“…In fact, patients with EMD at the time of the diagnosis, despite a lower International Scoring System (ISS) score, have poorer outcome regardless of first-line treatment (14). For de novo EMD patients who are eligible for stem cell transplantation, a triplet induction therapy approach (bortezomiblenalidomide-dexamethasone) is suggested, followed by high-dose melphalan with autologous haematopoietic cell transplantation, triplet consolidation therapy (bortezomiblenalidomide-dexamethasone), and maintenance treatment consisting of at least lenalidomide (15). Using thalidomide in the treatment of patients with EMD is controversial.…”