1 0 0 0 OA 日本アフェレシス学会雑誌
- 出版者
- 日本アフェレシス学会
- 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
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.
- 著者
- 大貫 順一
- 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.26, no.1, pp.101-120, 2007-02-28
- 著者
- 奥山 泰裕 山田 裕道 池田 志斈
- 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.27, no.2, pp.139-144, 2008-05-31
- 被引用文献数
- 1
Toxic epidermal necrolysis (TEN) is one of the serious drug eruptions with a high mortality rate. TEN also may cause severe consequences such as visual or respiratory disorders. Therefore, immediate diagnosis and initiation of correct therapy are most important. Apheresis therapy including plasma exchange for the treatment of TEN has been approved by the National Health Insurance System in Japan from 2006, due to the accumulation of successfully treated TEN patients with apheresis. In this study, we summarized the TEN patients treated with apheresis in our country, and discussed the timing to recruit the apheresis, as well as the mechanisms of apheresis. These examinations will provide an insight into a more appropriate treatment modality for the patients with TEN.
1 0 0 0 自己抗体と動脈硬化(<特集>老化とアフェレシス)
- 著者
- 松浦 栄次 小林 和子 小池 隆夫
- 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.23, no.2, pp.167-175, 2004-05-31
Oxidative stress is thought to be etiologically related to atherosclerosis. Experimental evidence clearly demonstrates the presence of oxidized LDL (oxLDL) in the intima of arteries and that it contributes to the initiation and progression of atherosclerotic lesions. We have recently demonstrated that oxLDL interacts with β2-glycoprotein I (β2-GPI) to form complexes and that these complexes circulate in the blood stream of patients not only with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) but also with other systemic inflammatory diseases. Our in vitro experiments showed that oxLDL quickly interacts with β2-GPI via specific ligands generated by oxidation, in which the negative charge of oxLDL is neutralized by the complex formed. It was also shown that β2-GPI/oxLDL complexes are taken up by macrophages via lgG anti-β2-GPI autoantibody-mediated phagocytosis. Thus, IgG immune complexes with oxLDL and β2-GPI are thought to be atherogenic and β2-GPI/oxLDL complexes maybe implicated as autoantigens relevant in autoimmune-mediated atherogenesis in APS patients. In contrast, a set of reports indicated that natural antibodies (mainly of the IgM class) derived from hyperlipidemic mice reduced the incidence of atherosclerosis in experimental models. Further study should elucidate whether IgG and/or IgM antibodies induced to oxLDL and β2-GPI/oxLDL complexes in APS are pathogenic or protective, or are an epiphenomenon in the development of arterial thrombosis.
- 著者
- 井上 昭彦 佐藤 尚文 尾形 敏郎 五十嵐 清美 野田 大地 良永 康雄 飯島 耕作
- 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.29, no.3, 2010-10-31
- 著者
- 金蔵 拓郎
- 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.27, no.2, pp.152-157, 2008-05-31
Granulocyte and monocyte adsorption apheresis (GCAP), an extracorporeal apheresis instrument using a column containing cellulose acetate beads designed to remove pathogenic granulocytes, was initially approved for the treatment of ulcerative colitis. We, as dermatologists, first assessed the therapeutic efficacy of GCAP in a patient with pyoderma gangrenosum which is often associated with ulcerative colitis and obtained remarkable results. We started the clinical study to assess the effectiveness of GCAP for treating various skin diseases attributable to activated neutrophils and investigated the mechanisms underlying the adsorption of pathogenic neutrophils. The effect of GCAP was assessed in 40 patients with neutrophilic dermatoses. The mechanisms by which the instrument adsorbs activated neutrophils were investigated using an in vitro mini-column system that mimics the GCAP. Of 39 patients with skin lesions, 32 (82.1%) benefited from GCAP. Mac-1 (CD 11 b/CD 18) expression on the peripheral neutrophils, increased compared to normal subjects, was reduced by GCAP. In the mini-column system, neutrophil adsorption was inhibited significantly by treating plasma with EDTA to inactivate complement and blood cells with anti-human CD 11b monoclonal antibody. Based on these results, we strongly propose GCAP as a useful treatment modality for skin disorders attributable to activated neutrophils.
