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PubMed1990

Clinical studies on adequate dosage of heparin during immunoadsorption with membrane plasmapheresis.

Heparin has been used as an anticoagulant during hemodialysis and plasmapheresis. Recently, immunoadsorption with membrane plasmapheresis (IAP) has been used for treatment of myasthenia gravis and multiple sclerosis. Ten patients were examined. Plasma heparin levels were studied before IAP, 30 minutes, 60 minutes, 90 minutes, and 120 minutes after the start of IAP, and at the end of IAP. Prothrombin time, activated partial thromboplastin time (APTT), bleeding time, plasma fibrinogen levels, fibrin degradation products, and complete blood count were measured pre- and post-IAP. In general, plasma heparin levels lower than 1 IU/ml during plasmapheresis are adequate. The dosage of 40 IU/kg/hr of heparin administered during IAP resulted in plasma heparin levels lower than 1 IU/ml. APTT pre-IAP (36.8 +/- 3.4 sec) were nearly one-half values of APTT post-IAP (6.2 +/- 9.70 sec). In conclusion, 40 IU per kg of the body weight per hour is an acceptable dose of heparin during IAP.

Heparin↗
PubMed1992

Role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating central nervous system disease that is associated with high morbidity and mortality. Although the recognition of ADEM may be facilitated by newer imaging techniques, the optimal treatment of this disease remains uncertain. We describe 4 patients with severe ADEM who responded to treatment that included intensive plasmapheresis. Two of the patients were in coma at the time that plasmapheresis was instituted, and all 4 patients made an excellent recovery. Immunologic studies revealed increased serum IgA levels, increased circulating immune complex levels as measured by the Raji cell assay, and decreased numbers of T and B cells prior to treatment of ADEM. These abnormalities improved following plasma exchange. Plasmapheresis appears to be effective in reversing the neuropathologic process in ADEM. The role of this treatment modality in ADEM requires further evaluation in controlled clinical trials.

Acute Disease↗
PubMed1984

Plasmapheresis combined with lymphocytapheresis and cytotoxic drugs as a therapeutic modality in neuroimmunological diseases.

Rebound after plasmapheresis is thought to be due to enhanced antibody and/or immune complex production. To prevent rebound a combination of steroids and immunosuppressive drugs has been used: lymphocytapheresis was employed in most patients, for not more than 10 sessions. 50 patients with myasthenia gravis, inflammatory myopathy, chronic dysimmune polyneuropathy and immune complex polyneuropathy have been treated and long-lasting benefits obtained in 35 patients. Rebound effects were observed only when cytotoxic drugs were not given or discontinued too soon. 8 patients who had been treated by plasmapheresis combined with steroids alone, after some recurrences of their disease, were switched to cytotoxic drugs, steroids and lymphocytapheresis combined with plasma exchange: in this group 7 patients eventually gained long lasting remissions. Our clinical experience strongly supports the hypothesis of a synergy between plasmapheresis and immuno-suppressive measures.

Autoimmune Diseases↗
PubMed1993

Treatment of amiodarone iodine-induced thyrotoxicosis with plasmapheresis and methimazole.

The present report illustrates the clinical and biochemical outcome in two amiodarone iodine-induced thyrotoxicosis (AIIT) patients submitted to plasmapheresis. Amiodarone was discontinued, and treatment with MMI (40 mg/day) was started. In addition, patients were submitted to two sessions of plasma-exchange, with a one-day interval between the two session. In both patients serum total T3 (TT3) and free T3 (FT3) concentrations decreased promptly but in contrast to the serum TT3, FT3 levels remained steadily above the normal range. A similar behaviour was observed for total T4 and free T4 plasma concentrations. Interestingly, a clearcut clinical amelioration was observed in both patients even before a reduction of circulating free thyroid hormone concentrations could be documented. In conclusion, our experience indicates that plasmapheresis may be useful in order to obtain a rapid amelioration of severe clinical picture of thyrotoxicosis, but cannot be considered as a definite therapy in AIIT. It should be considered that plasmapheresis is not devoid of risks and is also a very expensive procedure.

Aged↗
PubMed1996

[Plasmapheresis and immunopathogenetic aspects of toxic epidermal necrolysis].

Drug-induced toxic epidermal necrolysis (TEN) is a rare but frequently lethal disease of the skin and mucosa of unknown etiology. Toxic metabolic and immunologic products in the serum have been discussed as possible causes. For this reason we have performed plasmapheresis on these patients for many years. Results regarding survival and healing of the lesions are quite favorable, as exemplified with two patients described here who benefited from the procedure despite their old age. In order to gain insight into possible disease mechanisms that would explain the beneficial effect of plasmapheresis, we did immunohistochemical studies on these patients before and in one case after the procedure. In dermis and epidermis, there was a striking increase of macrophages and a relative increase of CD8-positive lymphocytes which might act as cytotoxic effector cells; these findings can be interpreted as the result of an immunological reaction. An increased staining of lesional epidermis with TNF alpha, as previously noted by other authors, should be interpreted with caution since necrotic cells are involved. On the basis of the clinical results, the pathogenetic considerations, and in view of the lack of specific treatment modalities, clinical studies concerning plasmapheresis for the treatment of toxic epidermal necrolysis are recommended, particularly since this procedure is widely available and generally well tolerated.

Aged↗
PubMed1997

[Papular mucinosis--successful therapy with plasmapheresis].

A 23-year old female patient presented with a 14 year history of cutaneous mucinosis. Her hands showed indurated edema with acrocyanosis and severe reduction in motility, while her face was red, edematous and revealed numerous small angiomas. In the sacral region, she had a large elevated skin colored plaque. On the basis of clinical findings, histology and further laboratory tests the disease was classified as papular mucinosis (lichen myxoedematosus). In view of the severe suffering of the young patient and an unsuccessful previous therapy with chloroquine, we decided to employ plasmapheresis as a single therapy. After the first treatment course there was an improvement in her skin condition. After a total of four plasmapheresis courses over a period of 18 months (total exchange volume of 38.4 l) there was an objective flattening of the plaques and reduction of the finger swelling. This case demonstrates that monotherapy of plasmapheresis is an effective mode of treatment for cutaneous mucinosis, a disease which is extremely difficult to treat.

Adult↗
PubMed2000

Double recurrence of FSGS after two renal transplants with complete regression after plasmapheresis and ACE inhibitors.

A patient who had undergone a first cadaveric donor kidney transplantation for idiopathic focal segmental glomerular sclerosis (FSGS), had an immediate recurrence of a biopsy-proven FSGS that eventually led to graft failure within 5 years from transplantation. The patient underwent a second cadaveric transplantation 10 months later. An immediate recurrence of a biopsy-proven FSGS occurred that was treated with two protracted cycles of plasmapheresis of seven months each, with the addition of an ACE inhibitor from the beginning. A complete and stable remission of FSGS was observed, which continues after more than 6 years from the end of plasmapheresis. The recurrence of FSGS after a second transplantation has a poor prognosis, but prolonged plasmapheresis treatment, by removing circulating factors altering glomerular permselectivity, and the addition of ACE inhibitors, through their potential interference with TGF-beta, might be synergistic in obtaining permanent remission.

Adult↗
PubMed1979

Treatment of Goodpasture's syndrome with plasmapheresis. A case report and review of the literature.

A case is reported of antiglomerular basement membrane antibody-induced Goodpasture's syndrome in which the patient required hemodialysis and was treated with immunosuppressive agents and plasmapheresis. A severe (80 per cent) cresentic lesion was reversed, and creatinine was stabilized at 2.5 mg/dl at one year follow-up. Earlier reports of therapy without plasmapheresis showed that 88 per cent of the patients would either die or require long-term hemodialysis. Fifteen other reported cases of Goodpasture's syndrome in which the patients were treated with plasmapheresis are reviewed. When reported, short-term follow-up showed that nine of these patients were alive without need of dialysis, five wee receiving dialysis, and only two had died. This suggests that plasmaheresis and immunosuppressive therapy may reverse the renal lesion in some patients with Goodpasture's syndrome.

Adult↗
PubMed1990

Effects of intraoperative plasmapheresis on blood loss in cardiac surgery.

Intraoperative platelet-rich plasmapheresis allows autotransfusion of fresh, undamaged platelets and clotting factors at the completion of the operation. To evaluate this technology, we randomly assigned 100 consecutive patients who were to undergo an elective coronary bypass procedure and had normal clotting studies into the experimental (plasmapheresis) or the control group. Characteristics of both groups were similar, including average age (61.4 years versus 61.3 years [experimental versus control group]), sex (78% male versus 74% male), preoperative weight (80.9 kg versus 80.2 kg), preoperative red cell mass (1,989 mL versus 1,890 mL), perfusion time (102 minutes versus 106 minutes), and coagulation studies. Both internal mammary arteries were used in 68% of the patients. All patients had preoperative and postoperative blood volume determinations and complete clotting studies. Sixty-two variables related to bleeding were analyzed. Strict indications for transfusion were a hemoglobin level less than 7 g/100 mL in patients younger than 70 years and a hemoglobin level less than 8 g/100 mL in patients older than 70 years. The group receiving intraoperative plasmapheresis had a significant reduction in operative red cell mass loss (1,050 +/- 43 mL versus 1,226 +/- 61 mL; p = 0.021), a reduction in the average homologous transfusion (0.67 +/- 0.15 unit versus 1.8 +/- 0.25 units; p = 0.0002), and an increase in the percentage of patients not requiring blood transfusions (66% versus 32%; p = 0.001). This technique is useful in reducing postoperative blood loss and homologous transfusions.

Blood Coagulation↗
PubMed1990

Effects of plasmapheresis on familial type III hyperlipoproteinemia associated with glomerular lipidosis, nephrotic syndrome and diabetes mellitus.

A 59-year-old woman, one of 5 cases with familial type III hyperlipoproteinemia reported at our clinic to date, had nephrotic syndrome and diabetes mellitus, but had neither coronary atherosclerosis nor xanthoma. A renal biopsy specimen revealed a massive cluster of foam cells containing apolipoprotein B and E in the mesangial region of the kidney. A restricted diet intake combined with lipid-lowering drugs such as cholestyramine, clinofibrate, and bezafibrate, in addition to methylprednisolone was not very effective in lowering serum triglyceride and cholesterol levels within physiological ranges. Therefore, plasmapheresis, using a dextran sulfate-cellulose column, was performed. Repeated plasmapheresis resulted in a marked decrease in both serum total cholesterol and triglyceride. A second renal biopsy specimen performed 2 years later revealed a marked reduction in foam cells with concurrent improvement in her nephrotic syndrome and glucose intolerance. These results suggest that familial type III hyperlipoproteinemia may be responsible for glomerular lipidosis resulting in nephrotic syndrome. They also indicate that plasmapheresis using a dextran sulfate-cellulose column is very effective in the removal of abnormal lipoproteins such as beta-very low density lipoprotein and intermediate density lipoprotein in a case of familial type III hyperlipoproteinemia.

Diabetes Complications↗
PubMed1992

Plasmapheresis for severe, unremitting, chronic urticaria.

Histamine-releasing autoantibodies have been identified in chronic idiopathic urticaria. 8 patients with severe disease and histamine-releasing activity in their sera underwent plasmapheresis. Symptoms were abolished for 2 months in 1 patient and for 3 weeks in another, 2 showed almost complete resolution of symptoms, 2 had temporary relief, and the other 2 showed little change. Further investigation in 4 of the patients showed significantly reduced skin-test responses to fresh post-exchange autologous sera after plasmapheresis compared with stored pre-exchange sera, but the response to intradermal histamine remained unchanged. Blood cellular histamine increased as in-vitro serum histamine-releasing activity fell after plasmapheresis. These results favour a pathogenetic role for histamine-releasing autoantibodies in patients with chronic urticaria.

Adult↗
PubMed1990

Effect of plasmapheresis therapy on circulating levels of pemphigus antibodies.

We determined the circulating levels of pemphigus antibodies in 11 patients with pemphigus treated with plasmapheresis, corticosteroids, and immunosuppressive drugs and in 11 patients treated with corticosteroids and immunosuppressive drugs alone. Three weeks after hospitalization, the average titer of pemphigus antibodies had decreased from admission levels by 83% in patients treated with plasmapheresis but by only 18% in the other group (p less than 0.05). These results suggest that plasmapheresis, in conjunction with high doses of corticosteroids and immunosuppressive drugs, reduces circulating levels of pemphigus antibodies more rapidly than conventional therapy.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Plasmapheresis for Guillain-Barré syndrome.

Guillain-Barré syndrome is the most common cause of paralytic illness in children. An 8-month-old infant presented with severe Guillain-Barré syndrome associated with parainfluenza 3 infection, was treated with plasmapheresis, and had complete recovery. Plasmapheresis can be technically difficult to perform in small children and infants, but should be considered. Controlled trials should be performed to determine the indications for plasmapheresis in children with Guillain-Barré syndrome.

Humans↗
PubMedDate not supplied

Plasmapheresis associated with a relapsing course in Guillain-Barré syndrome.

An 11-year-old boy with severe Guillain-Barré syndrome underwent 2 courses of 5 plasmaphereses. During each course his strength and respiratory function improved. Within 3 days of completing each course, his deficits worsened. After a prolonged plateau following the second course of plasmapheresis, he spontaneously improved and continues to recover gradually 11 months following the onset of symptoms. Plasmapheresis appeared to produce a fluctuating course in our patient. Prior to the availability of effective therapeutic intervention for Guillain-Barré syndrome, a natural course, characterized by recurrence of symptoms, predicted poorer recovery. Fluctuations related to plasmapheresis may not carry similar implications; the clinician should consider this phenomenon in assessing prognosis.

Child↗
PubMed2005

Intravenous immunoglobulins and plasmapheresis combined treatment in patients with severe toxic epidermal necrolysis: preliminary report.

Toxic epidermal necrolysis (TEN) is an acute drug-induced life-threatening disorder characterised by extensive epidermal exfoliation and high rate of mortality. Between October 2000 and April 2003, five severe TEN patients were evaluated using a specific TEN severity-of-illness scale (SCORTEN) and treated for the first time, with a combined therapy using Intravenous Human Immunoglobulins (IVIG) and plasmapheresis. The standardised mortality ratio (SMR) analysis ([Sigma observed deaths/Sigma expected deaths]x100) was applied to establish how IVIG and plasmapheresis treatment could reduce TEN patient mortality. The observed mortality was one out of five patients corresponding to 20%. The expected mortality based on SCORTEN was 3.319 corresponding to 66%. The SMR analysis revealed a 70% reduction in mortality (SMR=0.30; 95% confidence interval, 0.0-0.96). Our series show a low mortality rate (20%) related to the severity of the patients (66% expected mortality). The use of IVIG in association with plasmapheresis has a rational basis and may be effective in severe TEN patients.

Adult↗
PubMed2005

Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis.

Opsoclonus-myoclonus syndrome is a distinct neurologic disorder characterized by opsoclonic eye movements, multifocal myoclonus, and ataxia, traditionally described as "dancing eyes, dancing feet." A presenting sign in 2% of children with neuroblastoma, it usually heralds a favorable prognosis for the tumor. Although opsoclonus-myoclonus syndrome usually presents at initial diagnosis or relapse, there are reports of delayed presentation, usually a few months after diagnosis. This report describes a patient with ganglioneuroblastoma who developed recurrent symptoms of opsoclonus-myoclonus syndrome 9 years after completing treatment, without evidence of recurrent tumor. Believed to be autoimmune in origin, opsoclonus-myoclonus syndrome frequently responds to immunomodulatory therapies, such as steroids or intravenous immunoglobulin. This patient did not respond adequately to either agent, so plasmapheresis, a less commonly used modality in opsoclonus-myoclonus syndrome, was attempted. His symptoms resolved after he received therapy with a combination of plasmapheresis and steroids over a 1-year period. After being slowly weaned off all therapy, he has been symptom-free now for over 3 years. Armstrong MB, Robertson PL, Castle VP. Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis.

Combined Modality Therapy↗
PubMed2004

[Ethics, motivations of plasmapheresis donors].

For 50 years, the French Blood Transfusion Service has been developing ethical concepts of anonymous, voluntary and non-profit donation. The subject of this work is the study of ethical aspects and motivations of plasmapheresis donors. Three hundred donors were questioned on these subjects. The questionnaire was created after analysing the semi-orientated interviews of ten donors. The donors are male, aged over 35, with relatively high social and professional backgrounds. The main reason given for the first donation is the request by a relative or another person. With regards to further donations, solidarity with patients is mentioned as a reason. Even if the majority of the donors are aware of the ethical aspects of donation, over 50% of them accept to give plasmapheresis regardless of these principles. Plasmapheresis donors are primarily motivated by solidarity reasons. Thereby, they are fully active in the evolution of society.

Blood Donors↗