著者

Weber Maria Steffen Hans-M. Haupt Walter F. Becker Karin Brunner Richard Mahrle Gustav Borberg Helmut

出版者

日本アフェレシス学会

雑誌

日本アフェレシス学会雑誌 (ISSN:13405888)

巻号頁・発行日

vol.16, no.1, pp.115-125, 1997

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Extracorporeal elimination therapy of metabolic diseases may be taken into consideration if the substrate to be removed is present in the circulation in an elevated concentration and if the endogenous synthe-sis rate does not exceed the amount of re-moval. Systemic amyloidoses are characte-rized by the presence of specific protein pre-cursor molecules in the serum. Polymeriza-tion leads to the formation of fibrils which are deposited as amyloid in the extracellular space. Depending on the variables of the disease to be treated, it may generally be possible to delay its progression, achieve a halt in progression or even initiate regression of amyloid deposition. Two patients, a mother and her son, 54 and 25 years of age, with familial amyloidosis due to the transthyretin met30 variant (Por-tuguese type), after a disease duration of 9 and 6 years, respectively, received regular plasma exchanges in two-week intervals over a period of P/2 years. Patients' plasma was exchanged at volumes of 155% and replaced by 5% human albumin solution. Transthyretin levels were reduced by 50 - 70% during each plasma exchange procedure. Clinical, neurological, opthalmological, laboratory, histological, and electron microscopical examinations were performed prior to, and after one year of plasma exchange therapy. The overall results indi-cate only slight progression in the older pa-tient and stability in the younger patient. Athird patient, 56 years of age, with immunglobulin-type (AL) amyloidosis due to monoclonal gammopathy of unknown significance, with cardiac involvement and rapid deterioration of renal function under chemotherapy was treated initially by plasma exchange over a period of 1 1/2 years without further cytotoxic treatment, and subsequently by Ig adsorption using protein A or anti-IgG columns. Para-protein levels were reduced to 20-25% of the pretreatment values by plasma exchange on two consecutive days. Renal function, clinical and echo-cardiographical signs of cardiac failure remained stable. Our experience having patients with two different forms of systemic amyloidosis sufficiently is encouraging to discuss the possible therapeutic role of extracorporeal elimination in delaying the progression of amyloidosis.