[Advances in diagnosis and treatment of AL amyloidosis].
AL amyloidosis is a systemic disease characterized by extracellular amyloid deposition in tissues, causing progressive dysfunction of affected organs. The main clinical syndroms include nephrotic-range proteinuria with or without renal dysfunction, congestive heart failure, hepatomegaly and peripheral or autonomic neuropathy. The aim of therapy is the reduction of monoclonal light chains, by suppressing the underlying plasma cell dyscrasia. Recent therapeutic strategies involve intermediate-dose chemotherapy or high-dose melphalan supported by peripheral blood stem cell transplantation. Alternative therapeutic approaches include thalidomide, lenalidomide, iododoxorubicin, etanercept and rituximab. This paper reviews the pathogenesis, diagnosis and therapy of the AL amyloidosis, focusing on clinico-morphological symptoms of renal involvement, monitoring of treatment response and supportive therapy.
Zelichowski
Grzegorz
G
Wojskowy Instytut Medyczny w Warszawie, CSK MON, Klinika Chorób Wewnetrznych, Nefrologii i Dializoterapii, Poland. grzegorz@wim.mil.pl
Lubas
Arkadiusz
A
Wańkowicz
Zofia
Z
D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D006801:Humans; D007167:Immunotherapy; D007674:Kidney Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D009404:Nephrotic Syndrome / Q000209:etiology
D000682:Amyloid; D000970:Antineoplastic Agents