An Overview of Light Chain Multiple Myeloma: Clinical Characteristics and Rarities, Management Strategies, and Disease Monitoring.

Abdul Rafae,Mustafa N Malik,Seren Durer,Muhammad Abu Zar,Ceren Durer

doi:10.7759/cureus.3148

Abstract

Light chain multiple myeloma (LCMM) constitutes approximately 15% of patients with multiple myeloma (MM). It has a poorer prognosis when compared to immunoglobulin (Ig) G or IgA variant. We performed a comprehensive literature search on LCMM and identified a total of 390 articles. After a detailed screening, six studies involving a total of 1054 LCMM patients were included. A literature review revealed bone pain and renal failure as the most common initial sign and symptoms while extramedullary disease (EMD) was acquired later during the progression of the disease. Bortezomib has shown superior efficacy in LCMM patients over nonbortezomib regimens as demonstrated by better overall response rate (95.5% vs. 60%), progression-free survival (PFS) (25% vs. 9% at two years), and overall survival (OS) (24% vs. 9% at five years). Moreover, better PFS was seen, when bortezomib was used in combination with bendamustine compared to dexamethasone (95% vs. 25% at two years). Similarly, better OS (90% at two years) was observed with bortezomib in combination with bendamustine. Monitoring of disease should include serum free light chain levels, as literature review revealed that serum assays were more sensitive in indicating the disease and predicting PFS and OS as compared to urine assays. We provide presentation patterns, clinical rarities, management strategies including their efficacy, and disease monitoring in patients with LCMM in our review paper.

Highlights

BackgroundMultiple myeloma (MM) constitutes for 10% of all hematological malignancies and 1% of all malignancies [1,2]
The five-year overall survival (OS) and progression-free survival (PFS) among patients who initially presented with extramedullary disease (EMD) at diagnosis were found to be significantly lower when compared to patients who initially presented with MM confined to bone marrow (31% vs. 59% and 21% vs. 50%) respectively [14]
Author Year N S/S at initial presentation S/S Bone pain Renal failure Weakness EMD S/S developed during disease progression S/S > 3 Lytic bone lesions EMD Pleural effusion Anemia Hypercalcemia POEMS syndrome

Summary

Introduction

Multiple myeloma (MM) constitutes for 10% of all hematological malignancies and 1% of all malignancies [1,2] It is a malignant disease characterized by abnormal proliferation of plasma cells and monoclonal immunoglobulins or free light chains (FLC). The annual age-adjusted incidence in the United States is 4 per 100,000 population and has remained stable for years [6]. The five-year overall survival (OS) and progression-free survival (PFS) among patients who initially presented with EMD at diagnosis were found to be significantly lower when compared to patients who initially presented with MM confined to bone marrow (31% vs 59% and 21% vs 50%) respectively [14]. The median age in patients with MM at the time of diagnosis is 65 years [7, 15]. Bone disease is the major cause of morbidity in patients with MM and can be detected on skeletal radiographs, magnetic resonance imaging (MRI), or computed tomographic (CT) scans [10, 16]

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