AA amyloidosis complicating monoclonal gammopathies, an unusual feature validating the concept of "monoclonal gammopathy of inflammatory significance"?

Alexandre Terré, Magali Colombat, Alexandre Cez, Claire Martin, Carine Diet, Sabine Brechignac, Silvia Oghina, Diane Bodez, Stanislas Faguer, Léa Savey, Joris Galland, Jean-Jacques Boffa, Gilles Grateau, Arnaud Jaccard, David Buob, Sophie Georgin-Lavialle

Journal: International journal of clinical practice 2021;75(11):e14817

PMID: 34490695

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Abstract

INTRODUCTION

AL amyloidosis is caused by the proliferation of an immunoglobulin-secreting B cell clone. AA amyloidosis is a rare complication of chronic inflammation. However, some patients present with diseases combining monoclonal immunoglobulin production and chronic inflammation. The aim of this work was to describe cases of AA amyloidosis associated with monoclonal gammopathies.

PATIENTS AND METHODS

We reviewed all patients reported in French national amyloid centres presenting with AA amyloidosis and monoclonal gammopathy and performed a literature review. The quality of AA amyloidosis diagnosis and the causal relationship with monoclonal gammopathy were assessed.

RESULTS

In total, four patients from our centres and eight from the literature fulfilled the inclusion criteria. The haematological disorders presenting with monoclonal gammopathy were as follows: Waldenström macroglobulinaemia (n = 8), Schnitzler syndrome (n = 2), multiple myeloma (n = 1) and monoclonal gammopathy of undetermined significance (n = 1). Treatment strategies varied among the cases, with the treatment of the haematological disorder in 4 and anti-inflammatory treatment in 2.

CONCLUSION

Monoclonal gammopathies might be a rare and poorly known cause of AA amyloidosis. Such monoclonal gammopathies could be named "monoclonal gammopathies of inflammatory significance."

Address: Department of Internal Medicine, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, Paris, France.; Laboratoire d'Excellence GR-Ex, Institut Imagine, INSERM U1163, CNRS ERL 8254, Université Paris Descartes, Sorbonne Paris-Cité, Paris, France.; Department of Pathology, CHU Toulouse, Toulouse, France.; Department of Nephrology, Sorbonne University, Tenon Hospital, Paris, France.; Rheumatology Department, La Rochelle Hospital, La Rochelle, France.; Nephrology Department, Henri Mondor Hospital, Creteil, France.; Haematology Department, Avicenne Hospital, Bobigny, France.; Cardiology Department, Henri Mondor Hospital, National Reference Centre of Cardiac Amyloidosis, Creteil, France.; Cardiology Department, Centre Cardiologique du Nord, Saint-Denis, France.; Département de Néphrologie et Transplantation d'Organes, CHU de Toulouse, Toulouse, France.; Department of Internal Medicine, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, Paris, France.; Department of Internal Medicine, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, Paris, France.; Inserm UMRS_933, et laboratoire de génétique, Faculté de médecine, Sorbonne University, Trousseau Hospital, AP-HP, Paris, France.; Haematology Department, CHU Dupuytren, National Reference Center for AL Amyloidosis Limoges, France.; Department of Pathology, Sorbonne University, Tenon Hospital, Paris, France.

DOI: 10.1111/ijcp.14817

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MeSH Terms: Amyloidosis, Humans, Immunoglobulin Light-chain Amyloidosis, Monoclonal Gammopathy of Undetermined Significance, Multiple Myeloma, Paraproteinemias, Waldenstrom Macroglobulinemia