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PubMed2002

Resolution of severe Donath-Landsteiner autoimmune hemolytic anemia temporally associated with institution of plasmapheresis.

OBJECTIVES: To report a case of severe postinfectious autoimmune hemolytic anemia (AIHA) owing to the Donath-Landsteiner (DL) antibody resolving with plasmapheresis, and to review the pathophysiology of this underrecognized cause of pediatric AIHA and its potential susceptibility to plasmapheresis therapy. DESIGN: Descriptive case report. SETTING: A pediatric intensive care unit in a university children's hospital. PATIENT: A 5-yr-old Hispanic female had gastroenteritis followed by progressive intravascular hemolysis, initially attributed to acute postinfectious cold hemagglutinin (immunoglobulin M) disease. INTERVENTION: With no slowing in the rate of hemolysis, a continued need for frequent transfusions, and a lack of response to corticosteroid and intravenous immunoglobulin therapy, a 3-day course of plasmapheresis was administered. MEASUREMENTS AND MAIN RESULTS: The patient presented to an emergency department with an initial hematocrit of 22%, which fell to 12% by hospital admission. She received nine transfusions over 7 days, with her hematocrit reaching a nadir of 11% on the 5th day of hospitalization. Once plasmapheresis was initiated, she required no further transfusion. Analysis of serum from initial presentation demonstrated biphasic hemolysis, confirming the presence of the DL antibody. CONCLUSIONS: In AIHA, in which the direct antiglobulin test detects primarily C3 rather than immunoglobulin G, especially in children, the DL antibody must be considered. Confirming the diagnosis rapidly may be critical, especially in cases of severe hemolysis, because this may help direct therapy. A low titer of DL antibody can mediate severe intravascular hemolysis given its propensity to sensitize, detach, and rebind erythrocytes with changes in temperature in the microcirculation. However, given the transient and relatively brief production of the DL antibody in postviral illness, early clearance of the offending antibody may be possible with plasmapheresis, without the expectation for significant rebound antibody production, potentially decreasing the length of hospital stay and the need for transfusions.

Anemia, Hemolytic, Autoimmune↗

PubMed1986

Assessment of therapeutic plasmapheresis in demyelinating neurologic disorders.

Considerable controversy exists with regard to therapeutic efficacy of plasmapheresis in the immune-mediated demyelinating disorders of the peripheral and central nervous system: acute inflammatory polyneuropathy (Guillain-Barré syndrome), chronic inflammatory demyelinating polyneuropathy, and multiple sclerosis. In an effort to establish specific situations where plasmapheresis was of therapeutic value, we reviewed the experience at Southwestern Medical School, University of Texas Health Science Center at Dallas, and that published in the literature. In acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome), plasmapheresis may prevent progression of the illness, and it significantly increases the rate of recovery. For patients with chronic inflammatory polyneuropathy, plasmapheresis has produced clinical improvement in 50% of corticosteroid-refractory patients. The use of plasmapheresis in patients with acute or chronic progressive multiple sclerosis still remains controversial.

Adolescent↗

PubMed1997

Haemodynamic changes after high-volume plasmapheresis in patients with chronic and acute liver failure.

OBJECTIVE: To evaluate the haemodynamic changes during treatment with high-volume plasmapheresis in patients with chronic liver failure compared to patients with acute liver failure. METHODS: Haemodynamic measurements were performed with a Swan-Ganz catheter and thermodilution technique. High-volume plasmapheresis (mean plasma exchange of 8.6 litres) was performed in 11 patients with chronic and 16 patients with acute liver failure. RESULTS: In patients with chronic liver failure, systemic vascular resistance index was unaltered: 1193 +/- 494 dynscm-5m2 before treatment versus 1180 +/- 399 dynscm-5m2 after. Mean arterial pressure increased from 69 +/- 11 mmHg to 78 +/- 13 mmHg (P < 0.05) and cardiac output increased from 8.1 +/- 2.4 l/min to 8.9 +/- 2.4 l/min (P < 0.05) during high-volume plasmapheresis. In patients with acute liver failure, systemic vascular resistance index increased from 1154 +/- 628 dynscm-5m2 to 1614 +/- 738 dynscm-5m2 (P < 0.001). In this group mean arterial pressure increased from 78 +/- 16 mmHg to 95 +/- 10 mmHg (P < 0.001) and cardiac output decreased from 9.6 +/- 3.7 l/min to 8.2 +/- 2.9 l/min (P < 0.01). CONCLUSION: The hyperkinetic circulation in chronic and acute patients was differently affected by high-volume plasmapheresis. We suggest that in chronic liver failure both portosystemic shunting and chronic peripheral vasodilation may contribute to the hyperkinetic syndrome, whereas in acute liver failure a humoral factor which can be removed by high-volume plasmapheresis is a main contributor.

Acute Disease↗

PubMed1988

Effects of plasmapheresis on the plasma concentration of proteins used to monitor the disease process in multiple myeloma.

We studied the influence of plasmapheresis on the plasma concentrations of proteins (IgG, IgA, beta 2-microglobulin) used for estimation of tumour cell mass in patients with multiple myeloma. Simultaneously, the effects of plasmapheresis on plasma concentrations of proteins (CRP, alpha 1-antitrypsin, haptoglobin) used for the assessment of inflammatory processes were studied. Also, changes in the plasma concentration of protein HC, a recently described protein occurring both as a free protein and as an IgA complex, were studied. The influence of plasmapheresis varied for the different proteins. The plasma levels of IgG, IgA, CRP, alpha 1-antitrypsin, and haptoglobin decreased during plasmapheresis. The simultaneous reduction of the level of protein HC was smaller than that of IgG, IgA and haptoglobin. The plasma concentration of beta 2-microglobulin did not decrease during plasmapheresis. The recovery rates of the proteins were found to be approximately inversely related to their molecular weights.

Alpha-Globulins↗

PubMed2004

Therapeutic plasmapheresis in patients with severe hyperthyroidism in whom antithyroid drugs are contraindicated.

Four patients with Graves' disease in whom antithyroid drugs could not be used were treated by plasmapheresis preoperatively. On admission all patients had severe hyperthyroidism. All patients were treated by beta blockers, cholestyramine and inorganic iodine before plasmapheresis. Plasmapheresis course consisted of three sessions. Removed plasma was replaced by a synthetic colloid solution and human albumin other than fresh-frozen plasma. Plasmapheresis led to decreases in serum T3 concentrations >78-40% and free T4 concentrations >69%. Near-total thyroidectomy could be performed in all patients. Although screening coagulation tests were within normal limits, patients 1 and 4 experienced more blood loss than usual during the operative procedure. Plasmapheresis could be used as an alternative therapeutic option in the preoperative management of severe hyperthyroid patients with contraindications to antithyroid drugs. However, this is an invasive procedure and patients should be followed carefully for prolonged clinic/subclinic coagulopathy due to plasma exchange.

Adult↗

PubMed2007

Successful treatment of recurrent focal segmental glomerulosclerosis after kidney transplantation by plasmapheresis and rituximab.

A 22-year-old patient whose primary kidney disease was focal segmental glomerulosclerosis (FSGS) developed severe recurrence of proteinuria (up to 57 g/24 h) immediately after a haploidentic living donor kidney transplantation despite pre-operative plasmapheresis. The immunosuppressive treatment consisted of tacrolimus, mycophenolate mofetil, basiliximab and steroids. He underwent 10 plasmapheresis sessions in the first 3-week post-transplantation. In addition, he received 2 i.v. doses of rituximab (RTX) 600 mg (375 mg/m(2)) on days 7 and 15. Proteinuria decreased below nephrotic range at day 14 and serum creatinine returned progressively to normal values. A short course of oral ciclophosphamide (100 mg/j) was administrated between days 22 and 40 and three additional plasmapheresis sessions on days 34, 39 and 49. This strategy allowed obtaining sustained full remission of the nephrotic syndrome (NS) and excellent graft function, which persists over 2 years after transplantation. No notable adverse events related to RTX or plasmapheresis were observed. This case suggests that RTX associated with plasmapheresis may be an effective treatment of recurrent NS because of FSGS.

Adult↗

PubMed1998

Safety of plasmapheresis in the treatment of neurological disease.

BACKGROUND: Plasmapheresis is used as a treatment modality in several neurological disorders. Adverse reactions relating to vascular access, replacement fluid and anticoagulant use have been reported. AIMS: To establish the incidence of complications of therapeutic plasmapheresis in the treatment of neurological diseases. METHODS: A retrospective study was performed, in which case records of patients undergoing plasmapheresis for neurological indications between 1 April 1992 and 1 September 1996 were reviewed for fatalities and adverse reactions related to vascular access, transmission of Hepatitis B or C or Human Immunodeficiency Virus (HIV), fluid imbalance, allergic or febrile reactions, haematological complications, machine failure and citrate toxicity. RESULTS: In the study period, 1283 plasmapheresis procedures were performed on 73 patients with neurological disease. There were no fatalities, no cases of Hepatitis B or C or HIV transmission, no allergic or febrile reactions, thrombosis, bleeding diathesis or severe citrate toxicity. Infections related to venous access occurred in 0.5 per cent of procedures with septicaemia in 0.2 per cent of procedures (4.1% of patients). Less serious complications included hypotension in 1.2 per cent of procedures (11% of patients) and citrate toxicity manifesting as nausea, vomiting, cramping or paraesthesia in 1.2 per cent of procedures. Machine failure occurred in 0.2 per cent of procedures. CONCLUSIONS: Plasmapheresis is a safe procedure when performed for neurological indications by experienced personnel in a large pheresis unit. The most frequent adverse event is infection of the venous access site.

Adult↗

PubMed1981

Reticuloendothelial system Fc receptor function and plasmapheresis in systemic lupus erythematosus: a preliminary report.

Reticuloendothelial System (RES) Fc receptor-mediated immune clearance and levels of immune complexes were measured in patients with systemic lupus erythematosus undergoing plasmapheresis. RES clearance defects improved and disease manifestations and levels of circulating immune complexes decreased after plasmapheresis. Patients with initially abnormal reticuloendothelial system function, elevated levels of circulating immune complexes, and active acute illness appeared to respond to plasmapheresis. One patient with normal immune clearance, lacking measureable immune complexes, and manifesting chronic disease remained unchanged. Improvements seen following plasmapheresis were best maintained in patients receiving parenteral cyclophosphamide in the post-pheresis period. The correlation of clinical response, improved RES function, and decreased levels of immune complexes suggests the interaction of the latter two parameters in disease pathogenesis. Plasmapheresis has an important role as a research tool, and may be of clinical usefulness in a small number of patients with serious complications of systemic lupus erythematosus.

Adult↗

PubMed1992

Elimination of an artificial immune complex by plasmapheresis in rabbits.

The behavior of an artificial immune complex was investigated in 15 rabbits. The immune complex was labeled with iodine-125 (125I). The advantage was that the immune complex could not be metabolized without being eliminated by the kidney. This artificial immune complex was injected in a dosage of 0.8 microgram, in 2 rabbits, 5.0 micrograms in 4 rabbits, 10 micrograms in 3 rabbits, 20 micrograms in 2 rabbits, and 40 micrograms in 4 rabbits on two consecutive days. One group (n = 9) was treated with plasmapheresis: the other (n = 6) was a control group that did not undergo plasmapheresis. A miniaturized plasmapheresis system eliminated the immune complex very effectively with an average exchange volume of 95.7 ml per treatment. The increasing concentration of the immune complex in the blood before the next plasmapheresis treatment was probably an expression of mobilization of the immune complex from different organs. The animals of the plasmapheresis group were in better condition than the control group.

Animals↗

PubMed1999

Plasmapheresis in myasthenia gravis. A comparative study of daily versus alternately daily schedule.

OBJECTIVES: The aim of this study was to compare the efficacy of different protocols of plasmapheresis in the treatment of myasthenia gravis (MG). MATERIALS AND METHODS: We treated 30 MG patients with plasmapheresis on either a daily or alternately daily schedule for 5 consecutive sessions. Acetylcholine receptor antibody (AchRAb), serum proteins including albumin, globulin, immunoglobulin G (IgG), IgA, and IgM, and MG score were measured before and after the course of plasmapheresis in each group of patients. RESULTS: The mean percent reductions of serum proteins including IgA (81.5% vs 69.7%), IgM (95.6% vs 87.1%), and globulin (63.2% vs 50.1%) were significantly higher in the daily group. There were no significant differences in AchRAb and IgG levels after treatment between these 2 groups. However, the reduction of MG score was greater in the daily group. All the patients tolerated plasmapheresis well except for 2.7% of them who experienced hypotension. CONCLUSION: Our results suggest that daily plasmapheresis may be more effective in the treatment of patients with advanced MG.

Adult↗

PubMed1998

Immunoadsorption plasmapheresis in acute ataxic neuropathy.

Acute ataxic neuropathy is characterized by sensory ataxia and areflexia. There is no established treatment. We tried immunoadsorption plasmapheresis 15 days after the onset for a 46-year-old woman suffering from this neuropathy. She could not walk even with assistance because of sensory ataxia. A sural nerve biopsy revealed active axonal degeneration and loss of myelinated fibers. We tried 5 sessions of plasmapheresis during 2 weeks. She could walk with assistance 12 days after the beginning of the plasmapheresis treatment. It took 3 months for her to be able to walk over 5 m without assistance, and she had severe sensory ataxia over a 17 month follow-up period. Immunoadsorption plasmapheresis started within 2 weeks after the onset of acute ataxic neuropathy may have beneficial effects if the axonal degeneration is mild. The plasmapheresis, however, should be continued for a longer period. A double blind study is necessary to clarify the effectiveness of this treatment on acute ataxic neuropathy.

Acute Disease↗

PubMed1998

The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports.

A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).

Adult↗

PubMed2005

Protective antigen and toxin neutralization antibody patterns in anthrax vaccinees undergoing serial plasmapheresis.

Recipients of licensed anthrax vaccine (AVA; Biothrax) could serve as a source of hyperimmune plasma and immunoglobulin for therapy and prophylaxis. We measured serum antibodies during serial weekly to biweekly plasmapheresis in 38 individuals previously vaccinated with 4 to 27 doses of AVA. Immunoglobulin G (IgG) to protective antigen (PA) and toxin neutralization assay (TNA) antibody levels were highly correlated (r = 0.86930 and P < 0.0001 for anti-PA concentration versus TNA concentration). Significant decreases in antibody titer and concentration were observed over time when compared for the number of days from the last AVA injection (P < 0.0001 for both anti-PA and TNA concentration) and for the number of days from the first plasmapheresis (P = 0.0007 for anti-PA concentration and P = 0.0025 for TNA concentration). The rate of the decrease in total IgG concentration (half-life [t(1/2)] = 198.90 days after first plasmapheresis) was significantly less than the decrease in anti-PA IgG (t(1/2) = 63.53 days) (P < 0.0001), indicating that the reduction in anti-PA IgG was more likely due to natural decay than plasmapheresis. The time since the last injection and the time after initial plasmapheresis are important elements in considering an optimal schedule for collecting anthrax hyperimmune plasma. Good correlation between IgG to PA and TNA antibodies suggests that the anti-PA enzyme-linked immunosorbent assay can be used as a high-throughput screen for functional immune reactivity in donor plasma units.

Adult↗

PubMed1983

Plasmapheresis in the initial treatment of insulin-dependent diabetes mellitus in children.

Several factors indicate that autoimmune mechanisms may play a part in the aetiology of insulin-dependent diabetes mellitus. At the onset of the disease in 10 children (aged 11-16 years) plasmapheresis was performed four times over one to two weeks. Seventeen age-matched children with the same clinical features served as controls. The C-peptide concentrations at onset were the same in the two groups, but after one month the children treated with plasmapheresis had significantly higher values. This difference became even more pronounced after three, nine, and 18 months, both during fasting and at the maximum response to a standardised meal. The study group also had a significantly more stable metabolism, longer partial remission, and no higher insulin requirement. Of the 10 treated children islet-cell cytoplasmic antibodies were present in seven before plasmapheresis and in nine during treatment. The antibodies remained detectable in five and six out of nine patients at one and six months respectively after plasmapheresis. Although the mechanisms are obscure, plasmapheresis performed at the onset of insulin-dependent diabetes mellitus may help to preserve beta-cell function.

Adolescent↗

PubMed1985

Role of plasmapheresis in primary biliary cirrhosis.

Five patients with primary biliary cirrhosis and prolonged cholestasis underwent intensive plasmapheresis. The indications for plasmapheresis included intractable pruritus or hypercholesterolemia and xanthomatous neuropathy. Patients noted a rapid improvement of pruritus and fatigue which was sustained as long as plasmapheresis was continued. Cholesterol levels were lowered an average of 10.3 mmol/l and xanthomata were reduced in three of four patients. Two patients with painful neuropathy caused by xanthomata experienced relief of this symptom. The liver and spleen size were not affected by plasmapheresis, and activities of aminotransferases, alkaline phosphatase and titres of mitochondrial antibody remained unchanged. We conclude that plasmapheresis has a role in the therapeutic management of patients with advanced primary biliary cirrhosis who are disabled by the complications of pruritus, xanthomatous neuropathy, or hypercholesterolemia with xanthoma formation.

Adult↗

PubMed1982

Plasmapheresis: its value in the management of patients with antibodies to factor VIII.

12 plasmapheresis were carried out in 5 patients with antibodies to F VIII (3 haemaophilic antibodies; 2 spontaneous antibodies). Plasmapheresis led in all instances to a marked reduction of the antibody level there was a good correlation between the amount of plasma exchanged and the decrease of the antibody level. About 40 ml of plasma/kg body weight have to be removed to reduce the antibody level to half. In patients with low titre antibody who need treatment for serious bleeding, plasmapheresis is a more rapid and less expensive procedure than neutralisation of the inhibitor by high doses of F VIII. In one haemophiliac repeated plasmapheresis and subsequent high dose F VIII treatment eliminated the antibody within a short time. Plasmapheresis should always be considered when patients with antibodies to F VIII have to be treated because of severe bleeding.

Adolescent↗

PubMed1986

Effects of plasmapheresis and of hypoproteinemia on lung liquid conductance in awake sheep.

The Starling equation, which describes net transvascular liquid flow, does not include the possibility that a reduction in plasma protein concentration may have a direct effect on lung liquid conductance or microvascular protein permeability. Nevertheless, both effects have been reported. Since these results were not predictable, we wondered whether the changes were due to the decrease in plasma protein concentration or to the process by which protein depletion was accomplished (batch plasmapheresis which involves considerable handling of blood). To separate these factors, we did control (sham) and protein removal plasmapheresis in awake sheep by two plasmapheresis methods (batch and continuous-flow). We monitored pulmonary hemodynamics, measured lung lymph flow, and determined protein concentrations in lymph and plasma. We calculated or measured the protein osmotic pressures of plasma and lymph. After control plasmapheresis, lymph flow increased and lymph:plasma protein concentration decreased but had returned to baseline levels by 4 hours. After protein removal plasmapheresis, the changes persisted for 24 hours. However, lung microvascular conductance (filtration coefficient) was not increased, except during the first 4-hour period. The changes in lymph flow and protein concentration ratio are explained using a simple two-pore model. We conclude that, over the range studied, hypoproteinemia does not increase lung microvascular liquid conductance or protein permeability.

Animals↗

PubMed1992

Removal of phenytoin by plasmapheresis in a patient with thrombotic thrombocytopenia purpura.

Phenytoin removal by plasmapheresis was evaluated in a 17-year-old girl with thrombotic thrombocytopenia purpura. Free and total phenytoin concentrations were measured in the patient's serum and in the plasma removed by plasmapheresis. Plasmapheresis was performed on three separate days with the removal of 4.7%, 3.3%, and 2.7% of total body stores. Free phenytoin concentration was similar in both the plasma removed by plasmapheresis and the patient's serum. Plasmapheresis did not significantly alter the serum concentration of phenytoin; dosage adjustments of phenytoin are therefore unnecessary.

Adolescent↗