Treatment of Patients with Monoclonal Gammopathy of Clinical Significance

Abstract

Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of a monoclonal protein (M-protein) produced by a small amount of plasma cells. The majority of patients remain asymptomatic; however, a fraction of them develop clinical manifestations related to the monoclonal gammopathy despite not fulfilling criteria of multiple myeloma or other lymphoproliferative disorder. These patients constitute an emerging clinical issue coined as monoclonal gammopathy of clinical significance (MGCS). The mechanisms involved are poorly understood, and literature is scarce regarding management. The clinical spectrum involves symptoms related to renal, neurologic, skin, ocular, or bleeding manifestations, requiring a multidisciplinary approach. Treatment strategies rely on the basis of symptomatic disease and the M-protein isotype. In this review, we focus on MGCS other than renal, as the latter was earliest recognized and better known. We review the literature and discuss management from diagnosis to treatment based on illustrative cases from daily practice.

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

“…However, the treatment of most of these disorders is far from being standardized due to its heterogeneity and rarity. Despite the absence of solid evidence to make clinical decisions in most scenarios, the treatment possibilities for MGCS have recently been summarized [ 105 ]. The transfer of the patient to an experienced center offering multidisciplinary support or expert advice is recommended.…”

“…On the other hand, no standardized scores to assess the risk of progression have been reported to date. Few reports have attempted to describe the risk of progression from MGCS to symptomatic MM or other lymphoproliferative disorders [ 105 ].…”

Acquired bleeding disorders

“…Recently, is has been suggested that a benefit‐to‐risk approach should be made in patients with MGUS. As AVWS in the context of MGUS is potentially life‐threatening, myeloma treatment regimens can be considered 32 . A careful evaluation of the bleeding symptoms, including preoperatively, can help to decide if the patient would benefit from the initiation myeloma treatment.…”

What about (MG)US? Towards tailored testing in monoclonal gammopathies

“…9 A further layer of complexity in monoclonal gammopathies is the existence of very rare, nonmalignant, albeit severely disabling entities, such as light-chain amyloidosis or renal, neurologic or myopathic disease for which the acronym "MGCS" (monoclonal gammopathy of clinical significance) was coined. 2 These entities are typically characterized by their underlying low-burden plasma cell dyscrasias and by a wide spectrum of clinical symptoms. This highlights the need for more sensitive laboratory tests both with respect to confirm presence of a monoclonal gammopathy and for monitoring treatment response.…”