Abstract

Although there are similarities in the treatment paradigms between AL amyloidosis and multiple myeloma, there are also fundamental differences. A similarity is of course the use of anti-plasma cell drugs in both diseases; however, the most serious mistake a hemato-oncologist can make is to use the same treatment schedule in dosing and frequency in AL amyloidosis patients as in multiple myeloma patients. AL amyloidosis patients with >10% bone marrow plasma cell infiltration in particular are at risk of receiving a more intensive treatment than they can tolerate. This difference in dosing and frequency is true for many anti-clonal drugs, but it is most apparent in the use of high-dose melphalan and autologous stem cell transplantation. While in multiple myeloma in the age group of ≤70 years, more than 80% of patients are fit enough to receive this intensive treatment, this is the case in less than 20% of AL amyloidosis patients. A similarity is the alignment in the goal of treatment. Although in AL amyloidosis has long been recognized that the goal should be complete hematological remission, this has become more apparent in multiple myeloma in recent years. A common goal in the coming years will be to evaluate the role of minimal residual disease to improve survival in both diseases.

Highlights

  • AL amyloidosis is a disease belonging to the group of plasma cell neoplasms

  • We describe some of the similarities and differences between the two diseases

  • The different prognostic systems clearly reflect the differences between the two diseases: MM is more dependent on the dynamics of its plasma cell clone and AL amyloidosis is more dependent on misfolding-prone free light chain (FLC) and, the involvement of internal organs and their amyloid load

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Summary

Introduction

AL amyloidosis is a disease belonging to the group of plasma cell neoplasms. (smoldering) multiple myeloma (MM), plasma cell leukemia, solitary plasmacytoma of the bone, and extra-osseous plasmacytoma. These diseases all have separate definitions, as published and defined by the International Myeloma Working Group (IMWG) in 2014 [1]. The disease is defined by a plasma cell clone of any size in the bone marrow combined with the presence of extra cellular deposits of amyloid in visceral organs and related organ dysfunction. Hemato 2021, 2 of AL amyloidosis patients being treated with myeloma-based treatment regimens that they cannot tolerate. This may result in an increased toxicity profile and in severe side effects. We describe some of the similarities and differences between the two diseases

Epidemiology
Prognostic Systems
Plasma Cell Characteristics
IgM AL Amyloidosis
Treatment
Intensive Therapy with High-Dose Melphalan and Autologous SCT
Treatment of ASCT-Ineligible Patients
Findings
Conclusions
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