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Monoclonal gammopathy related disorders. The presence of a monoclonal immunoglobulin in serum or urine reflects the existence of an abnormal clone of B cells. In case of large tumor mass, patient need treatment. Sometimes, the clone is nearly undetectable, but gammopathy is associated with complications not directly related to tumor cells but to the presence of the monoclonal immunoglobulin. Most often, lesions result from the deposit of monoclonal immunoglobulin, organized or not. Other mechanisms include autoantibody activity, immune complex formation or deregulation of the complement system. Finally, in some cases, the physiopathology remains to be determined. The downstream complications of gammopathies are protean and can affect various organs, mainly the kidney, the skin and the peripheral nervous system. A delayed diagnosis may be life-threatening or functional. A rigorous clinical examination, and appropriate explorations are therefore necessary in order to put in place a suitable therapy, targeting most often the underlying clone., Competing Interests: Les auteurs décla rent n’avoir aucun lien d’intérêts.

Competing Interests: Jean-Paul Fermand déclare n’avoir aucun lien d’intérêts.

Monoclonal gammopathy of renal significance. The term "monoclonal gammopathy of renal significance" regroups all renal disorders caused by a monoclonal immunoglobulin (Ig) secreted by a nonmalignant B-cell clone. However, a small clone can synthesize a very toxic protein, producing devastating renal and sometimes systemic damages. The spectrum of renal diseases in MGRS is wide. Renal lesions are classified according to the localization, either glomerular or tubular, and to the pattern of ultrastructural organization of Ig deposits. Physico-chemical characteristics of pathogenic monoclonal Ig are probably involved in their propensity to deposit or precipitate in the kidney, as illustrated by the high rate of recurrence of renal lesions after kidney transplantation if the underlying clone is not suppressed. Early diagnosis and efficient chemotherapy are mandatory to improve renal prognosis and patient survival, particularly in patients with systemic lesions such as AL amyloidosis., Competing Interests: Les auteurs déclarent n’avoir aucun lien d’intérêts.

The finding in serum and/or urine of IgG, IgA or a monoclonal light chain must prompt a systematic search for a myeloma, a solitary plasmocytoma or an immunoglobulins amyloidosis. Clinical and experimental data suggest that some monoclonal immunoglobulins can remain stable after several years of existence, but at the time of diagnosis there is no criterium to assert with certainty that a monoclonal immunoglobulin will or will not be complicated by a malignant proliferation of plasmocytes. Nothing but a prolonged follow-up enables a diagnosis of "benign" monoclonal gammapathy to be made.

Some progress in the treatment of multiple myeloma (MM) has been recently obtained due to a better knowledge of prognosis factors and of indications of conventional chemotherapies. More over, two new therapeutic approaches appear very promising, including the use of alpha-interferon and the introduction of high dose chemotherapy protocols used alone or in combination with total body irradiation and hematopoietic transplantation. All available therapeutic strategies and their place in the management of patients with MM are successively discussed.