血浆置换治疗原发性巨球蛋白血症的临床研究

利用ＣＳ３０００血细胞分离机对４例巨球蛋白血病行血浆置换疗法，每次置换量为２５００－３０００ｍｌ，共进行１７次血浆转换。血浆置换前后测定下述指标：Ｈｂ、Ｈｃｔ、ＷＢＣ、ｐｌａｔ、ＥＳＲ、血清免疫球蛋白。结果显示每次血资料置换后血清ＩｇＭ平均下降３３．３％。血浆转换量与血清ＩｇＭ水平呈负相关。     

血浆置换联合化疗治疗原发性巨球蛋白血症

原发性巨球蛋白血症 (PMG)是 B淋巴细胞恶性增生性疾病。随着我国老龄化社会的到来 ,此种疾病的发病率亦有明显增加。当前治疗仍以化疗为主 ,但对血清中高水平的 Ig M的下降较缓慢 ,疗效不满意。本文对单用化疗无效的 6例 PMG患者采用血浆置换 (PE)联合化疗治疗取得了明显的疗效。现报告如下。1　资料与方法1 .1 　 临床资料1 997年 4月～ 1 999年 1 2月我科共收治 6例PMG患者 ,男 2例 ,女 4例 ;年龄 41～ 67岁。首发症状为 :乏力 4例 ,伴头晕、消瘦、腹泻、心悸各 1例 ;鼻、牙龈出血 1例 ,间断长期发热 1例 ,检查 :6例皆有不同程度的…     

瘤可宁与血浆置换联合治疗华氏巨球蛋白血症初步观察

作者对５例华氏巨球蛋白血症患者采用ＨｅｍｏｎｅｔｉｃｓＶ５０血细胞分离机行血浆置换（ＰＥ）治疗，同时予小剂量瘤可宁，所有病人经１疗程血浆置换后获部分缓解，３例病人停止ＰＥ治疗半年到１年后复发，经再置换后缓解，随访１５～６４月病情稳定，故ＰＥ配合瘤可宁是治疗华氏巨球蛋白血症的有效手段。     

血浆置换治疗高胆红素血症

治疗性血浆置换 (ＴＰＥ)在临床上应用很广泛 ,但用于治疗慢性重症肝炎或肝炎后肝硬化引起的非梗阻性高胆红素血症报道较少。我们对 10例高胆红素血症患者行治疗性血浆置换 ,临床疗效显著。1　材料与方法1.1　一般资料　 1997年 9月～ 1999年 12月 ,10例患者中 ,男 6例 ,女 4例 ,平均年龄 4 1± 11岁。其中慢性重症肝炎 6例 ,肝炎后肝硬化 4例。共行 15次ＴＰＥ ,其中 5例作 2次ＴＰＥ。治疗前总胆红素 311.2± 4 5 .2 μｍｏｌ/Ｌ ,直接胆红素 2 13.3± 33.8μｍｏｌ/Ｌ ,间接胆红素 97.9± 18.5 μｍｏｌ/Ｌ。1.2　设备与材料　ＣＯＢＥＳ…     

血浆置换术治疗巨球蛋白血症1例

l998～2001年．我院应用血浆置换术(TPE)治疗巨球蛋白血症1例,取得良好效果。现报告如下。     

血浆分离与化疗2例原发性巨球蛋白血症

原发性巨球蛋白血症是一种少见的淋巴样浆细胞恶性增生性疾病,作者应用血浆分离与化疗两例患者取得一定疗效,现报告如下: 1 病例介绍     

血浆置换治疗难治性病毒性肝炎高胆红素血症

血浆置换治疗难治性病毒性肝炎高胆红素血症金龙，郑水根血浆置换治疗从八十年代起作为一种肝脏暂时性的支持措施用于暴发性肝功能衰竭的患者［１］。我们将此疗法用于治疗对常用药物无反应的难治性病毒性肝炎高胆红素血症患者，获满意效果。现介绍如下。材料与方法一、一...     

老年巨球蛋白血症的治疗

老年巨球蛋白血症是指老年患者血中出现大量单克隆IgM并发贫血、出血、高黏滞综合征等一系列临床表现。因本病首先被瑞典学者Waldenstrom发现 ,故用其名命之 ,本病患者约占巨球蛋白血症全部患者的 2 5 % ,属恶性浆细胞病范畴。1　诊断本病的诊断依据是老年患者血清中出现单克隆     

小剂量血浆置换治疗慢性肝炎高胆红素血症临床研究

慢性肝炎高胆红素血症是目前肝病治疗的难题之一,且易进展至重型肝炎.加速黄疸消退,阻断其转为重型肝炎,是改善本病预后的关键.     

Status of plasmapheresis for the treatment of toxic epidermal necrolysis in Japan

This study describes the efficacy of plasmapheresis for the treatment of toxic epidermal necrolysis (TEN), as reported in Japan. TEN patients treated with plasmapheresis were collected from Japanese literature. The type of plasmapheresis, number of sessions, efficacy of plasmapheresis, and present outcome were examined. Forty-seven TEN patients treated with plasmapheresis have been reported in the literature: 19 men and 28 women with ages ranging from 1 to 96 years (mean 50.3 years). Twenty-five of these treatments included simple plasma exchange (PE), 13 included double filtration plasmapheresis (DFPP), and one included PE and DFPP. The number of plasmapheresis sessions ranged from 1 to 6 and the mean number of sessions was 3.1. The efficacy of plasmapheresis for the treatment of TEN was as follows: excellent, 30 cases; good, 8 cases; fair, 3 cases; no effect, 5 cases; and unknown, 1 case. The rate of effectiveness was 80.9%; eleven patients died; and the mortality rate was 23.4%. In summary, plasmapheresis, including both PE and DFPP, were found to be effective treatments for Japanese patients with TEN, who had been unresponsive to corticosteroids, and for those who show severe clinical manifestations.     

A case report of plasmapheresis in the treatment of acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system associated with significant morbidity and mortality. High-dose corticosteroid administration has been considered the mainstay of treatment for ADEM; however, some patients with ADEM are refractory to steroid therapy. We report a case of a 17-year-old man suffering from ADEM who did not respond to corticosteroid therapy, but who exhibited a dramatic recovery with plasmapheresis. He became comatose, requiring ventilatory support, and exhibited abnormalities of some brainstem reflexes prior to treatment. He underwent sequential courses of plasma exchange therapy for three days. Plasma exchanges were carried out with concomitant continuous hemodiafiltration (CHDF) to control intracranial pressure by stabilizing pH, plasma Na+ concentration, and colloid osmotic pressure. After plasma exchanges, his reflexes and level of consciousness gradually improved. Eleven months after this treatment, he had only minimal neurological deficit that did not interfere with any of his activities of daily living. The efficacy of plasmapheresis for ADEM has not yet been established. Plasmapheresis may be indicated for ADEM, not only for patients with severe disease in whom high-dose corticosteroid treatment has failed, but also as first-line treatment for ADEM. Early initiation of plasmapheresis appears to be associated with moderate to marked improvement. Early recognition and early treatment of ADEM are thus of paramount importance.     

Approach to acute renal failure with multiple myeloma: role of plasmapheresis

Multiple myeloma is commonly complicated by renal failure, which limits therapeutic options and aggravates the prognosis. In myeloma the most common cause of advanced renal failure is myeloma kidney, where light chains produced by tumor cells precipitate and impede kidney function. We suggest that plasma exchange is an appropriate intervention in advanced renal failure from multiple myeloma, and support this notion with two case reports. The two elderly individuals had advanced chronic and acute renal failure, and recovered after the removal of large quantities of light chains by existing membrane separation techniques. We also present an algorithm to approach renal failure and myeloma.     

Long-term clinical outcomes of synchronized therapy with plasmapheresis and intravenous cyclophosphamide pulse therapy in the treatment of steroid-resistant lupus nephritis

In recent years, the vital prognosis of the patients with lupus nephritis (LN) has improved dramatically as a result of steroid therapy and the administration of immunosuppressants including cyclophosphamide, but recurrent cases and complications associated with these therapies are a concern. In this study, a long-term retrospective evaluation was performed over a period of five years concerning the clinical characteristics, remission rate and relapse rate by dividing 38 patients with LN into three groups receiving either plasmapheresis (PP), intravenous cyclophosphamide pulse therapy (IVCY), or both PP and IVCY (synchronized PP-IVCY) as the treatments added to steroid therapy. The complete remission rates of PP, IVCY and PP-IVCY were 5/9 (55.6%), 8/16 (50.0%) and 9/13 (69.2%), respectively. The relapsing rates of PP, IVCY and PP-IVCY were 3/9 (33.3%), 3/16 (18.8%) and 1/13 (7.7%), respectively. Synchronized PP-IVCY therapy might be superior to PP or IVCY in achieving complete remission of LN, and in minimizing the risk of relapse of impaired renal function.     

Successful plasmapheresis in alveolar hemorrhage associated with systemic lupus erythematosus

Abstract

The case of an 18-year-old Japanese man with systemic lupus erythematosus (SLE) complicated by alveolar hemorrhage is described. The patient presented with fever, butterfly rash, and polyarthralgia, and was diagnosed with SLE. He suddenly developed alveolar hemorrhage during steroid pulse therapy. Treatment with plasmapheresis was initiated, with prompt clearing of the chest radiograph. This experience suggests that the prompt initiation of plasmapheresis should be considered for SLE patients with life-threating alveolar hemorrhage resistant to conventional immunosuppressive therapies.     

Successful plasmapheresis in alveolar hemorrhage associated with systemic lupus erythematosus

The case of an 18-year-old Japanese man with systemic lupus erythematosus (SLE) complicated by alveolar hemorrhage is described. The patient presented with fever, butterfly rash, and polyarthralgia, and was diagnosed with SLE. He suddenly developed alveolar hemorrhage during steroid pulse therapy. Treatment with plasmapheresis was initiated, with prompt clearing of the chest radiograph. This experience suggests that the prompt initiation of plasmapheresis should be considered for SLE patients with life-threating alveolar hemorrhage resistant to conventional immunosuppressive therapies. Received: December 27, 2000 / Accepted: March 26, 2001     

No V(H) somatic hypermutation was detected in B-cells of a patient with macroglobulinemia due to splenic marginal zone lymphoma

B-cell diseases are classified on the basis of the normal differentiation stages. We report here a case of a patient with a long history of leukocytosis, splenomegaly without lymphadenopathy, and hyperviscosity symptoms. Clinically, the patient's diagnosis was leukemic Waldenstrom macroglobulinemia. Chromosomal analysis revealed translocation t(2;7)(p11;q22) along with disease progression. Death occurred from pulmonary infection at 46 months after the initial presentation. At autopsy, malignant lymphocytes were found in the marginal areas of the spleen with spreading to the bone marrow and the liver. The histologic findings were consistent with splenic marginal zone lymphoma. We examined the sequences of the immunoglobulin V(H) gene in cells from the initial peripheral blood and from the spleen at autopsy and found that the sequences were identical and had no somatic hypermutation. Macroglobulinemia can occur in various B-cell disorders, including splenic marginal zone lymphoma, even with the transformation of unmutated B-lymphocytes.     

血浆置换成功治疗毒蕈中毒性肝病1例

患者男性，47岁，无慢性肝炎病史，无长期大量饮酒史，近期无服用药物史。2005年11月13日与其他4人进食野蘑菇，10h后均出现呕吐、腹泻等胃肠道中毒症状。其中2人次日出现尿黄，并逐渐出现皮肤、巩膜黄染。第4天收治入当地医院治疗，诊断为急性胃肠炎、黄疸性肝炎。肝功能检查：总胆红素（TBil）289μmol／L，直接胆红素（DBil）211．9μmol／L，丙氨酸氨基转移酶（ALT）449U／L，天冬氨酸氨基转移酶（AST）200U／L。电解质和肾功能均正常。经输液治疗，胃肠道症状好转出院。第6日出现头晕、嗜睡，并逐渐出现意识障碍（肝性脑病）。2005年11月20日收治入我科。     

Theoretical basis of pathogenic substance removal during plasmapheresis

This article explains theoretical considerations of prescribing plasmapheresis. It is important to consider several factors in prescribing apheresis: (i) the properties of pathogenic substances, such as molecular weight, distribution volume, compartment, and production rate; (ii) therapeutic conditions such as the processed plasma volume and the frequency of processing; and (iii) the patient's condition, such as active bleeding or infection. A substance's molecular weight determines whether it can be removed using a particular membrane filter. Substances with a small distribution volume and low production rate are removed. Consequently, the processed volume per total plasma volume is related to the single-session efficacy. Nevertheless, even frequent therapy cannot reduce the total pool of a substance within the body if movement from outside the vessels is slow. Active bleeding or infection might be exacerbated by modalities other than simple plasma exchange with fresh plasma supplementation because such therapies cannot replenish substances other than albumin.     

The nonspecific clearance function of the reticuloendothelial system in patients with immune complex mediated diseases before and after therapeutic plasmapheresis

Summary The nonspecific clearance function of the reticuloendothelial system (RES) in six patients with immune complex mediated systemic vasculitis was determined by the evaluation of the disappearance rate of technetium 99m labelled microaggregated human serum albumin colloid (MHAC) injected IV before and after therapeutic plasma exchange. Three patients with systemic lupus erythematosus (SLE) and one patient with immune complex vasculitis (ICV) exhibited a significant clinical improvement after plasmapheresis which was paralleled by an accelerated MHAC elimination rate following plasma exchange therapy. One patient with ICV and unresponsive to plasma exchange showed delayed MHAC elimination. In one patient with myasthenia gravis (MG), the elimination rate was not altered by plasmapheresis. The data obtained indicate that nonspecific clearance of the RES may be one effect of plasma exchange therapy in patients with immune complex mediated diseases.