Use Of Therapeutic Plasma Exchange Research Articles

Current insight into thrombotic thrombocytopenic purpura

During the last three decades knowledge regarding the pathophysiology of thrombotic thrombocytopenic purpura (TTP) has dramatically increased. The discovery of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) deficiency in a subset of patients with TTP has been an important milestone. Apart from this, the use of therapeutic plasma exchange has reduced mortality rates in TTP from 80-90% to 10-20%. Nevertheless, TTP remains a possibly lethal disorder, in which early recognition of symptoms remains extremely important. In the last few years some interesting new insights into TTP have arisen. Firstly, promising reports on rituximab in the treatment of refractory and relapsing cases of TTP have been published. Secondly, risk stratification by means of ADAMTS13 deficiency and ADAMTS13 antibodies might lead to a more tailored approach in treating TTP patients.

Transplantation-associated thrombotic microangiopathy: effect of concomitant GVHD on efficacy of therapeutic plasma exchange

Factors predictive of response to plasma exchange (PE) in treatment of transplantation-associated thrombotic microangiopathy (TA-TMA) are generally poorly understood. To determine any clinical or laboratory factors predictive of response to PE in treatment of TA-TMA, we retrospectively reviewed all 11 cases of TA-TMA treated with PE at out institution between December 2001 and March 2008. Response to PE was correlated with associated clinical conditions, grade of TA-TMA, TA-TMA index and lactate dehydrogenase (LDH) level at diagnosis. Overall response to PE was 27%, with three complete responses (CRs) and eight non-responses (NRs) seen. Response to PE was significantly associated with the absence of acute GVHD at TA-TMA diagnosis, with three CR in four patients without active acute GVHD, and NR in seven patients with acute GVHD (P=0.024). There was no significant association seen between response to PE grade of TA-TMA (P=0.179), TA-TMA index (P=0.25) or LDH measurements (P=0.31). Our experience in use of therapeutic PE for management of TA-TMA suggests that PE may indeed be effective in the treatment of this disorder, depending on the clinical circumstance in which it develops. PE is potentially efficacious in the absence of active acute GVHD, and ineffective when acute GVHD is manifest.

Management of familial hypertriglyceridemia during pregnancy with plasma exchange

Hypertriglyceridemia-induced pancreatitis is a serious complication of familial dyslipidemias. Hormonal influences during pregnancy can compromise otherwise controlled lipid levels in women with familial hypertriglyceridemia and predispose to pancreatitis leading to increased morbidity in both mother and fetus. We report the successful use of therapeutic plasma exchange (TPE) in the management of hypertriglyceridemia during pregnancy resulting in avoidance of pancreatitis and delivery of a healthy term infant. Thirteen TPEs were performed from 19 to 36 weeks gestation to maintain tight control of triglyceride levels.

Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis

Limbic encephalitis (LE) is a neurological syndrome usually presenting in a paraneoplastic form. Recently many cases were reported with no concurring neoplasia, presenting with specific antibodies for voltage-gated potassium channel or for neuronal membrane antigens. Anti-glutamic acid decarboxylase (GAD) antibodies act against GABAergic receptors of the central nervous system. These antibodies were found in coeliac disease serum and in neurologic patients. We are reporting a case of a 21-year-old coeliac woman manifesting complex multiple-daily partial drug-resistant seizures for 7 years. The diagnosis was of a non paraneoplastic limbic encephalitis, unresponsive to high dose cortisone and IGIV infusion. The use of therapeutic plasma exchange (TPE) has resulted in an improvement in symptoms with quite a long disease-free period of time. When the frequency of the procedures was decreased, seizures appeared again and, after suspension of TPE, the clinical status worsened. The role of TPE in the treatment of LE still has to be defined.

Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature

Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fatal, central nervous demyelinating disease characterized by a rapid fulminant clinical course. Successful management requires early diagnosis, aggressive management of cerebral edema, and immunosuppression. Therapeutic plasma exchange (TPE) is infrequently used and commences after initial management fails. A 31-year-old man presented with right arm weakness, whose symptoms rapidly progressed to hemiplegia and aphasia. The patient was initially managed with glucocorticosteroids. Decompressive craniotomy and brain biopsies were performed when his intracranial pressure increased. Brain biopsy findings were consistent with AHLE. Mycoplasma pneumonia immunoglobulin G and immunoglobulin M serologies revealed recent infection. Despite surgical and medical management, he decompensated on Day 11, and TPE was initiated. The patient received a total of 10 TPE treatments. On the fourth day of TPE treatment, he was extubated. Twenty-one days after TPE began, he was ambulating with near normal muscle strength and was discharged. Four months after initial presentation, the patient has normal strength and is working full-time. AHLE has a fulminant course requiring accurate and rapid diagnosis. Successful therapy requires aggressive management of intracranial pressure and immunosuppression. Two other reports of AHLE document successful management with TPE. Each of these patients survived with minimal neurologic impairments. Given the likely immune-mediated nature of this disease, combined treatment of steroids, surgery, and TPE may lead to shorter hospital stays and improved neurologic outcomes. Clinical studies are needed to further study the effect of TPE on neurologic outcome in AHLE.

Goodpasture Syndrome

Goodpasture Syndrome

New directions in plasma exchange.

This review examines the literature published on therapeutic plasma exchange during 2002. The review was performed by searching Medline for pertinent articles. One hundred thirty articles were identified, of which 11 are reviewed. During the period, reviews of the use of therapeutic plasma exchange for managing Guillain-Barré syndrome and myasthenia gravis were published. A large randomized trial of the use of plasma exchange to treat sepsis also appeared. Finally, a large case series of the use of plasma exchange in Wegener granulomatosis was published. The literature confirms the use of plasma exchange for Guillain-Barré syndrome but suggests that inadequate evidence exists to support its use for long-term improvement in myasthenia gravis. The study of patients with severe sepsis suggests that plasma exchange may benefit a subset of patients, those with abdominal infections. Finally, plasma exchange for Wegener granulomatosis with severe renal dysfunction appears not to offer any benefit over immunosuppressive therapy.

Therapeutic plasma exchange in recurrent focal segmental glomerulosclerosis following transplantation.

Focal segmental glomerulosclerosis (FSGS) recurs in 30% of renal allograft transplants with graft loss in half of the cases. A humoral factor may be implicated. We report on the use of therapeutic plasma exchange (TPE) in 11 patients with recurrent FSGS post transplantation. Medical records from 1989-2000 were reviewed for 11 adults transplanted for biopsy proven FSGS. Ten patients developed proteinuria (x: 6.1 g; range: 3-40 g/24 h) within 2 months of transplantation. In 1 patient, proteinuria (4 g) occurred 2 years post transplantation. Biopsy in six patients revealed early recurrent FSGS, while in five, suspected recurrence was based on clinical findings. Each patient received 5-11 TPEs (x: 6) with the COBE Spectra, daily or on alternate days with 2.5-3.5 L 5% albumin as the replacement fluid. In four, FFP was included because of coagulopathy. All received immunosuppression (IS) during and after TPE. A persistent drop in 24 h urine protein (U.P.) was observed in 10/11 patients. Seven had >70% drop in 24 h U.P. following the course of TPE, while three had a reduction of 45-50%. No change occurred in 1 patient. Follow-up (9 months-5 years) of seven patients has shown a persistent U.P. of <1 g with successful allograft survival. In these patients, TPE appeared effective in early recurrent FSGS. The decrease in U.P. may result from combined TPE and IS. Although the disease is designated in category III by the ASFA, TPE should be considered early when FSGS recurrence is established.

Extracorporeal immunoadsorption with protein A: technical aspects and clinical results.

AbstractThe use of therapeutic plasma exchange (TPE) for removal of large molecular‐weight substances form patients' circulation is limited by the method's non‐selectivity and non‐specificity. In order to remove the target amount of pathogenic substance, usually amounting to less than 1 g per liter of plasma, one will also remove 60‐80 g of proteins, some of which are essential. A further disadvantage of the method is the need for substitution fluids, which at least in some countries are expensive and of limited availability. Furthermore, there is a (low) risk of disease transmission.To overcome these limitations, the principle of affinity chromatography used in biochemistry was applied in medicine to develop the Excorim Extracorporeal Immunoadsorption System in the beginning of the 1980s [1].

Ethical lessons learned from the use of therapeutic plasma exchange in neurologic disease.

Conflicts of interest are inherent in the practice of medicine, particularly in the conduct of clinical research. Such conflicts can often be identified and even resolved by the application of ethical principles as an integral part of such research. The opportunity to participate in a series of controlled clinical trials of therapeutic plasma exchange (TPE) in diverse neurologic disease provided an insight into some of the ethical dilemmas posed by such conflicts of interest. Chief among these was the recognition that informed consent is a precious though fragile and multidimensional concept. In the last analysis, ethics is the "business of being human"; respect for its guidance helps to ensure the success of clinical research.

The use of therapeutic plasma exchange for treatment of acute polyradiculoneuropathy

The use of therapeutic plasma exchange for treatment of acute polyradiculoneuropathy

The use of therapeutic plasma exchange for treatment of acute polyradiculoneuropathy

The rationale for therapeutic plasma exchange (TPE) in acute polyradiculoneuropathy (APRN) is based on the presence of circulating antibodies which cause demyelination of the peripheral nerves. This study included 24 patients, most having a neurological defect of grade 4, classified into groups A and B. Group A was treated with immunosuppressive drugs only, while group B was treated by a combination of immunosuppressive drugs and TPE. The average volume of plasma removed during a single TPE procedure was 2.95 L, and totalled 21 L overall. The efficacy of the TPE treatment was assessed on the basis of changes in the neurological deficit grade, and in electrophysiological parameters. It was shown that improvements were significantly better and faster in patients treated by a combination of immunosuppressive drugs and TPE, and when TPE was started within the first 7 days after the onset of the disease.

Complications of therapeutic plasma exchange: a recent assessment.

The use of therapeutic plasma exchange, particularly for management of patients with neurological disorders, has steadily increased in our hospital since 1991, thus highlighting the need for continuing quality assurance of these services. We have reviewed the hemapheresis records of all 381 therapeutic plasma exchange procedures performed on a total of 63 patients from January 1991 through December 1992. Patients were referred for therapeutic plasma exchange for the following indications: acute Guillain-Barre syndrome, 31 patients (49.2%); chronic inflammatory demyelinating polyneuropathy, 15 patients (23.8%); myasthenia gravis, five patients (7.94%); paraproteinemic neuropathy, five patients (7.94%); thrombotic microangiopathies, six patients (9.52%) and Goodpasture syndrome, one patient (1.6%). Overall, 89% of patients were treated for neurological disorders. Complications of plasma exchange were noted during 17% (n = 65) of procedures involving 49% (n = 31) of patients treated. Approximately 91% of complications were classified as mild (55.4%) or moderately severe (35.4%) and did not prevent successful completion of the procedure. These were largely related to use of citrate-containing anticoagulants and additionally may have reflected the autonomic instability of many of the neuropathy patients. All four (6.15%) severe complications and one of two fatalities were related to the use of central venous access catheters. We conclude that therapeutic plasma exchange can be performed safely with acceptable toxicity caused by mild and moderately severe complications. Major complications may be minimized by careful management of central venous catheters when required for access.

Therapeutic Plasma Exchange: Dermatologic Applications

Therapeutic plasma exchange has already been applied to a surprisingly wide range of patients with dermatologic disease who were refractory to conventional therapy. In most instances plasma exchange has been used to remove circulating autoantibodies and immune complexes, but it has also been useful in removing drugs, toxins, and harmful metabolic products. The rationale for the use of therapeutic plasma exchange and its potential side effects are discussed. Details are given regarding its specific use in collagen vascular diseases and other dermatologic disorders.