Sessions Of Plasmapheresis Research Articles

Photopheresis of a less than 10-kg child with acute graft versus host disease accompanied with hyperbilirubinemia: A case report

Hematopoietic stem cell transplantation is a curative treatment for many hematologic malignancies with its most important side effect being graft-versus-host disease (GVHD). Herein, we present a 3.5 year-old male with weight of 9.8 kg with acute GVHD (grade IV gastrointestinal and cutaneous) who did not respond to the first line therapies (corticosteroids). Thus, the patient was a candidate for extracorporeal photochemotherapy (ECP). Due to the hyperbilirubinemia, two sessions of ECP every week as well as one session of plasmapheresis 24 h before each ECP session were performed (Spectra™Optia® apheresis system). The procedures were performed successfully without any side effects and the GVHD manifestations of skin and GI responded perfectly to the treatment after 12 and 14 sessions of ECP, respectively. According to the results, it seems that ECP could be successfully performed in even less than 10-kg patients.

Desensitization with the Use of an Antibody Removal–Free Protocol in ABO-Incompatible Kidney Transplant Recipients with a Low Anti-A/B Antibody Titer

BackgroundDesensitization for ABO-incompatible (ABOi) kidney transplantation mainly comprises removal of antibodies with the use of apheresis and suppression of antibody (Ab) production with the use of rituximab. This study aimed to estimate the outcomes of ABOi kidney transplantation with the use of an Ab removal–free protocol to avoid complications associated with apheresis. MethodsA total of 32 de novo consecutive adults who underwent ABOi living-donor kidney transplantation were retrospectively evaluated. Our protocol for ABOi recipients was stratified and fixed according to the anti-A/B Ab titer at baseline before desensitization. Desensitization was performed before transplantation with 0–4 sessions of plasmapheresis or double-filtration plasmapheresis and 1–2 administrations of rituximab at 100 mg/body. Graft outcomes, anti-A/B Ab titer, and plasma fibrinogen level were compared between the Ab removal (n = 21) and Ab removal–free (n = 11) groups. ResultsBetween the Ab removal and Ab removal–free groups, the graft loss rate (4.8% vs 0.0%; P = 1.0), acute rejection rate (19.0% vs 0.0%; P = .14), and serum creatinine level (1.74 vs 1.40 mg/dL, P = .53) were similar. The anti-A/B Ab titer was maintained at a low level until postoperative month 12 in both groups. The plasma fibrinogen level on the operation day was significantly lower in the Ab removal group than in the Ab removal–free group (163.4 vs 250.2 mg/dL; P < .001). ConclusionsDesensitization with the use of an antibody removal–free protocol for ABOi kidney transplant recipients with a low anti-A/B Ab titer can maintain excellent graft outcomes and avoid postoperative bleeding risk.

Distribution of Anti-ABO Immunoglobulin G Subclass and C1q Antibody in ABO-incompatible Kidney Transplantation

IntroductionTo investigate the correlation between serum anti-ABO immunoglobulin G (IgG) and IgG subclasses, anti-ABO IgG subclasses were measured by flow cytometry (FCM) in ABO-incompatible (ABOi) kidney transplant recipients. We also evaluated baseline anti-ABO C1q antibody. MethodBaseline anti-ABO IgG titers were measured by both FCM and column agglutination technique methods in 18 ABOi kidney transplant recipients. The mean florescence intensity (MFI) ratios of baseline anti-ABO IgG subclasses and anti-ABO C1q antibody were obtained by FCM and followed-up after rituximab treatment, each plasmapheresis (PP) session, and kidney transplantation. Correlation between the values of IgG subclass and total IgG titer was analyzed. ResultsThe baseline MFI ratios of total IgG, IgG1, IgG2, IgG3, and IgG4 were 202.46, 62.41, 30.01, 1.04, and 1.13, respectively. The MFI ratios of IgG1, IgG2, and total IgG measured at baseline and pre-PP were positively correlated with the baseline ABO titer was measured using the column agglutination technique. The numbers of PP sessions to reach the target titer were correlated with the baseline IgG and IgG1 levels. IgG1 and IgG2 as well as total IgG were removed effectively after serial PP. Anti-ABO C1q antibody was neither detected nor correlated with total IgG and any IgG subclasses. ConclusionsOur findings suggest that IgG1 and IgG2 are the dominant IgG subclass in ABOi kidney transplant recipients. Baseline levels of IgG1 and IgG2 were correlated with baseline total IgG titer. However, anti-ABO C1q antibody was not detected in the present study.

Steroid Withdrawal Using Everolimus in ABO-Incompatible Kidney Transplant Recipients With Post-Transplant Diabetes Mellitus

BackgroundThe effectiveness of everolimus (EVR) for ABO-incompatible (ABOi) kidney transplantation is unknown. We evaluated outcomes of conversion from steroid to EVR in ABOi kidney transplant recipients. MethodsWe performed a retrospective observational cohort study of 33 de novo consecutive adult ABOi living donor kidney transplant recipients. Desensitization was performed using 0 to 4 sessions of plasmapheresis and 1 to 2 doses of 100 mg rituximab according to the anti-A/B antibody titer. ABOi recipients were administered a combination of tacrolimus, mycophenolate mofetil, and methylprednisolone. Diabetic patients were converted from methylprednisolone to EVR at 1 to 15 months post-transplantation to prevent diabetes progression. Graft outcomes, hemoglobin A1c (HbA1c) levels, and cytomegalovirus infection rates were compared between the EVR (n = 11) and steroid (n = 22) groups. ResultsMean postoperative duration was 814 and 727 days in the EVR and steroid groups, respectively (P = .65). Between the 2 groups, graft survival rate (100% vs 95.5%, P > .99), acute rejection rate (9.1% vs 18.2%, P = .64), and serum creatinine levels (1.46 mg/dL vs 1.68 mg/dL, P = .66) were comparable. Although HbA1c levels were elevated in the steroid group (5.47%, 5.87%; P = .003), no significant deterioration was observed in the EVR group without additional insulin administration (6.10%, 6.47%; P = .21). Cytomegalovirus infection rate was significantly lower in the EVR group than in the steroid group (18.2% vs 63.6%, P = .026). ConclusionConversion from steroid to EVR in ABOi kidney transplant recipients maintained excellent graft outcomes and avoided diabetes progression and cytomegalovirus infection.

Management of Plasma Cell-Rich Acute Rejection in Living-Related Kidney Transplant: Role of Proteasome Inhibitor.

Plasma cell-rich acute rejection is an aggressive form of acute rejection that occurs late after transplant and is usually resistant to standard antirejection therapy. This study reports the safety, efficacy, and outcomes of plasma cell-rich acute rejection after treatment with bortezomib, a proteasome inhibitor, in 10 patients after a first living-related renal transplant. Plasma cell-rich acute rejection was diagnosed using the 2007 Banff classification. The treatment protocol for plasma cell-rich acute rejection included methylprednisolone (500 mg/kg), 7 sessions of plasmapheresis, antithymocyte globulin (3-5 mg/kg/day for 10 days), rituximab (2 doses at 375 mg/m2), and bortezomib (1 cycle at 1.3 mg/m2). The mean age of recipients and donors was 23.70 ± 11.39 and 37.30 ± 12.82 years, respectively. The mean time to plasma cell-rich acute rejection was 3.1 ± 2.5 years. The mean serum creatinine level at rejection was 4.8 ± 2.7 mg/dL. After treatment, serum creatinine decreased to 3.3 ± 1.8 mg/dL. Serum creatinine levels at 1-year and 2-year follow-up were 3.0 ± 2.3 and 3.3 ± 0.9 mg/dL, respectively. There was 1 graft failure due to recurrence of glomerulonephritis/de novo glomerulonephritis. No significant adverse effects were noted in the patients. Bortezomib successfully reverted plasma cell-rich acute rejection and stabilized graft function, with patients showing 2-year graft survival after rejection of 90%. Bortezomib-based treatment was successful in reverting plasma cell-rich acute rejection and stabilizing graft function, with graft survival of 90% at 2 years. Further studies with large cohorts and randomized trials with or without bortezomib will help in better evaluation of its efficacy, safety, and outcomes.

Capillary leak syndrome as a complication of antibody-mediated rejection treatment: a case report.

We report a case of capillary leak that developed during treatment of antibody-mediated rejection in a kidney transplant recipient. A 53-year-old female transplant recipient experienced an increase in serum creatinine from 1.1 to 1.8mg/dL. Antibody-mediated rejection was diagnosed by graft biopsy. She was treated with five plasmapheresis sessions (on alternate days with albumin replacement), five doses of immunoglobulin (5g/dose at 100mg/kg), a single dose of rituximab (500mg), and four doses of bortezomib on days 1, 4, 7, and 10 (1.72mg/dose at 1.3mg/m2 body surface area). During treatment, edema, slight diarrhea, pancytopenia, hypoalbuminemia, peripheral neuropathy, and postural hypotension were noted. Despite control of liquids, she presented with edema progressing to an increase of more than 10kg body weight. Prerenal acute graft dysfunction associated with hypotension was diagnosed on day 12, heart failure or other infectious complications being discounted. On day 13, daily hemodialysis was prescribed, and a stable volume status was reached after five hemodialysis sessions. On day 20, the patient recovered diuresis and the edema and diarrhea abated, but she remained on chronic hemodialysis. After excluding other causes of distributive shock, the diagnosis of capillary leak syndrome was based on the presence of hypotension, generalized edema, and hypoalbuminemia in the absence of significant proteinuria. The concomitant presence of diarrhea, peripheral neuropathy, and pancytopenia, suggest a possible causal role for bortezomib. Awareness by clinicians of capillary leak syndrome associated with bortezomib-based treatment of AMR is paramount, despite its rarity.

Role of Plasmapheresis in the Management of Acute Kidney Injury in Patients With Multiple Myeloma: Should We Abandon It?

The aim of the current study was to determine whether plasmapheresis in combination with chemotherapy could significantly remove free light chains (FLC) in multiple myeloma (MM) patients with acute kidney injury (AKI) and therefore improve renal recovery and patient survival. During the study period, 29 patients with MM and AKI presented to our unit and were treated with two different therapy modalities (plasmapheresis with chemotherapy or bortezomib). At the end of treatment, a significant decrease of FLCs was present in the group treated with plasmapheresis compared to the bortezomib group. Patients treated with plasmapheresis had similar survival compared to patients treated with bortezomib. There was a significantly higher decrease of FLCs and longer survival in patients treated with three or more plasmapheresis sessions than in patients treated with two plasmapheresis sessions. Plasmapheresis therapy still remains a useful and effective method in the treatment of AKI in MM patients. Plasmapheresis significantly reduces FLCs compared to bortezomib especially with higher number of plasma exchange sessions but it must be combined with other chemotherapy agents in order to prolong renal recovery and therefore patient survival.

Potential role of plasmapheresis in severe cytomegalovirus infection with ongoing immunemediated hemolysis and low complement level

Symptomatic cytomegalovirus (CMV) infections are rare in patients with no previous history of immunosuppressive therapy or condition. A 33-year-old woman with type-2 diabetes, endstage renal disease (ESRD) and unexplained lower extremity weakness was admitted with failure to thrive, ongoing hemolysis, low haptoglobin and C3 levels and suppressed IgM and IgG globulin fractions. Multiple cultures and serologic workup for autoimmune disorders remained negative. CMV viral titer by serum polymerase chain reaction (PCR) revealed massive elevation at 9.2 million copies/mL. Initial CMV IgG titer was positive, IgM titer remained consistently negative and CMV was recovered from cerebrospinal fluid, as well. She received treatment with I.V. ganciclovir, CMV immune globulin (CytoGam) and three sessions of plasmapheresis (PLEX). The extreme viremia without neutropenia, lack of IgM antibody reconversion and CMV-mediated hemolysis with a low complement level were all very unusual features. Timely initiation of PLEX may have contributed to recovery

P029 A successful case of stem cell transplant after desensitization of a strong donor-specific antibody

A 71 year old female was initially diagnosed with myelodysplastic syndrome (del 5q) which progressed to AML one year later. Patient had pancytopenia, experienced bruising, and needed blood transfusions. Allogeneic stem cell transplant was recommended and she presented to the University of Chicago Medicine for allo-stem cell transplant work up. This patient had one potential related donor (sibling), who was a 9/10 match (mismatch at HLA-A). When examining the patient’s HLA antibodies, it was found that she was highly sensitized, and anti-HLA-A24 was a donor specific antibody (DSA). A search for matched unrelated donors was conducted but no suitable donors were found. It was decided to accept her sibling and desensitize the patient prior to transplant. The desensitization protocol consisted of plasmapheresis (PP), with IVIG (100 mg/kg) given after each PP. The initial MFI of the DSA was >18,000 and showed evidence of rebound after each PP. As a result, PP was performed daily or every other day, with A24 MFI measurement before and after each PP (Figure). While the strength of A24 decreased over the course, a total of 18 sessions of PP were needed to decrease the strength to <2500, our laboratory’s cutoff for predicting a negative crossmatch. Flow cytometric crossmatch was performed post 19th PP, and the T cell result was negative. The patient received conditioning with a regimen of fludarabine/melphalan/campath and a final PP (20th). The transplant occurred on 11/29/2016 and showed donor engraftment two weeks later. The post-transplant antibody tests indicate that HLA-A24 became undetecTable 8 days after transplant (12/7/2016) and remained negative 5 days later (12/12/2016). The patient is currently stable and under follow-up. While desensitizing a strong DSA may require a large number of PP, it is nevertheless possible, and can be free of PP-associated complications. The protocol described here may be useful to remove strong DSA in patients with no other option than a DSA-positive donor.Download : Download high-res image (161KB)Download : Download full-size image

P085 Lung re-transplant with repeated mismatch HLA antigens

Avoidance of previous donor mismatched HLA antigens is often considered for lung re-transplant due to concerns of potential HLA sensitization and memory responses. However, this approach may result in the exclusion of a large number of potential donors from consideration. Recently, we successfully performed a re-transplant with a history of multiple repeated mismatches. A 30+ year old male with a past medical history of cystic fibrosis underwent two previous bilateral lung transplants in August 2004 and in November 2010. Both allografts failed due to CLAD/BOS that developed after respiratory viral infections. The patient also subsequently developed detectable class I and II HLA antibodies with a cPRA > 88% in May 2016. Among these antibodies, strong anti-A1, B7 and DQ2 were first donor HLA specific. The second donor shared an A3-B18-DR4 haplotype with the first donor but not the recipient. No HLA antibodies specific to this A3-B18-DR4 haplotype antigens or to any other mismatched HLA antigens of the second donor were detected. A third lung transplant became urgently necessary in October 2016. Adding his previous donors’ mismatched HLA antigens to the unacceptable HLA list resulted in a cPRA > 96%. Therefore, a personalized listing strategy was developed based on discussions between the HLA lab and the transplant program. A deceased donor became available in November 2016. The virtual crossmatch revealed that the donor had no unacceptable HLA antigens based on HLA antibody detection. However, this donor carried the A3-B18-DR4 haplotype which was a repeated mismatch haplotype of the previous two donors. Based on a risk and benefit assessment, this donor was accepted as the source of lungs for the third transplant. The patient received 5 plasmapheresis sessions post-transplant to prevent antibody mediated rejection. Frequent post-transplant HLA antibody monitoring has not detected development of donor specific antibodies to the third donor and the new allograft functions well. Lung re-transplant can be performed with a cross of a repeated HLA mismatch provided that the recipient has no detectable HLA antibodies specific to the current donor. Our preliminary findings obtained from our study conducted on UNOS lung transplant data supports this conclusion. An assessment of risk and benefit of crossing the repeated mismatch HLA antigens should be done on a case-by-case basis.

Clinical analysis of single filtration plasmapheresis using continuous renal replacement therapy machines in kidney transplantation.

BackgroundPlasmapheresis has become an essential element of kidney transplantation (KT). In the present study, we report clinical outcomes of filtration plasmapheresis using continuous renal replacement therapy machines with a single filter for the first time in Korea.MethodsWe retrospectively analyzed six patients who underwent filtration plasmapheresis for KT in our center; plasmapheresis was performed using the Plasmaflex (Baxter®) with a TPE 2000 filter set (Baxter®) in our hemodialysis unit. Five percent albumin was used as the replacement fluid, and intravenous immunoglobulin G was administered after each plasmapheresis session. The target preoperative ABO isoagglutinin titer was less than 1:8.ResultsFiltration plasmapheresis was performed in four patients for ABO-incompatible KT, one for antibody-mediated rejection after KT, and the last one for positive T cell crossmatch. Altogether, 46 sessions of plasmapheresis were performed. ABO isoagglutinin titers successfully declined to or below the target level in all patients, and all patients successfully received KT with no significant antibody titer rebound. Acute antibody-mediated rejection and positive T cell crossmatch were well treated with filtration plasmapheresis, and no patient required fresh frozen plasma infusion for coagulopathy. There were one episode of hypotension and three of hypocalcemia. No patients experienced bleeding, infection, or allergic reaction.ConclusionFiltration plasmapheresis was effective and safe. Although our result is from a single center, our protocol appears to be promising.

Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

A autoimmune Pulmonary Alveolar Proteinosis (PAP) patient with persistent disease underwent 3 Whole Lung Lavages (WLLs), 10 plasmapheresis sessions and further 3 WLL, from October 2004 to May 2007.

Ablative Therapy in 2016 for Small Renal Masses

The survival rate of kidney graft after kidney transplantation has been improved following the introduction of current immune suppression drugs. However, the risk of recurrence or de novo glomerulonephritis still remains. Most forms of glomerular disease can recur after transplantation [1] but different forms of glomerulonephritis have widely different allograft outcomes. Whereas IgA nephritis recurs in up to one third of transplanted patients, this is not associated with adverse effects on graft survival. In contrast, recurrent focal segmental glomerulosclerosis (FSGS) has an unfavorable prognosis [2] and so early detection and appropriate treatment can be crucial. We are presenting here a patient with primary FSGS who developed acute kidney injury and heavy proteinuria in the first week post-transplant that responded completely to multiple plasmapheresis sessions and four doses of Rituximab.

Role of low plasma volume treatment on clinical efficacy of plasmapheresis in neuromyelitis optica.

BACKGROUND:Neuromyelitis optica (NMO) is an autoimmune demyelinating disease preferentially targeting the optic nerves and spinal cord. Plasmapheresis (PP) is an effective adjunct therapy in severe NMO attacks. The recommended minimum plasma volume to be treated per session of PP is equivalent to total plasma volume (TPV) of the patient.AIM:To study the effect of lower plasma volume treated in patients with NMO on clinical efficacy of plasmapheresis in comparison to minimum recommended volume.METHODS:This retrospective study was done on acute NMO patients who were managed with PP at our center. Patients who had 5 sessions of PP, spread over 10 days, were included. Clinical outcome was defined as per predefined criteria.RESULTS:24 patients who underwent PP for acute NMO met our inclusion criteria. Females (age; mean (SD) 33.7 (11.2) years) were more common (n = 18). The minimum recommended plasma volume (PV) that was supposed to be treated per patient during entire acute therapeutic period was 195.5 (14.6) mL per kilogram-body-weight (kg-bw). We treated lower plasma volume (mean (SD) 112.7 (17.0) mL per kg-bw); the difference was significant (P < 0.05). The volume treated was same across the gender (P > 0.05). Significant clinical improvement was observed in 79% of patients (n = 19) after 6 months. There were no significant differences in volume of plasma treated, between patients who had moderate and marked improvement; also, who did, and did not have significant clinical improvement (P > 0.05; for both).CONCLUSIONS:Plasmapheresis is a safe and efficient add-on therapy in NMO, especially in steroid-resistant cases. Although the volumes of plasma treated during acute plasmapheresis were less than recommended minimum volumes, majority of patients had significant clinical improvement.

Compromiso cerebrovascular agudo en síndrome hemolítico urémico: descripción de dos casos pediátricos

To report two pediatric cases of D+HUS with severe neurological involvement due to cerebrovascular disease, and review available literature. Two previously healthy 15- and 21-month-old children debuted with seizures and impairment of consciousness within the first week of a D+HUS. Both presented hypertension, severe acute renal failure, and focal motor deficit. One child showed significant improvement in neurologic status after five sessions of plasmapheresis. Brain magnetic resonance showed in the first child multiple bilateral infarcts of small vessels and lesions of white matter. In the second patient, large bilateral infarcts on both middle cerebral arteries territories were identified. One year after the acute event, both children showed functional impairment; The first patient evolved with language delay and spastic hemiparesis; the second patient with spastic quadriparesis, epilepsy with poor seizure control and marked functional impairment. Although most of the children with D+HUS and brain involvement do not have long-term sequelae, cerebrovascular disease in the acute period causes permanent damage, and in addition to the management of electrolyte disturbances, hypertension, and renal failure, therapies directed at specific pathophysiological mechanisms that trigger vascular compromise may improve prognosis.

Clinical case of using discrete plasmapheresis in infant with early neonatal sepsis and hemolytic disease

Currently in the literature there are insufficient data on the use of efferent hemocorrection methods in neonatal practice. The basic principle of this method is the removal of plasma containing endotoxins and exotoxins and other pathological substances, replacing it with donor plasma, colloid and crystalloid solutions. The therapeutic effect of plasmapheresis includes detoxification, anti-inflammatory and immunomodulatory effects by removing toxins and removal of circulating immune complexes, inflammatory mediators, and activated structures of the complement system. Discrete plasmapheresis in neonatal practice may be used in any body mass of the patient. This article describes a clinical case of successful application of discrete plasmapheresis in full-term newborn baby is in critical condition. The severity was due to severe early neonatal sepsis, development of multiple organ failure and accompanying RH-conflict. After two sessions of plasmapheresis was observed a positive clinical effect in reducing intoxication, systemic inflammatory response syndrome. Reducing the level of bilirubin is allowed to avoid the operation replacement blood transfusion.

Baseline anti-blood group antibody titers and their response to desensitization and kidney transplantation

In recent years, immunological barriers historically considered as absolute contraindications to transplantation are being reevaluated. One such barrier is the ABO blood group incompatibility. With better understanding of immunological mechanisms and effective various regimens for controlling it, ABO-incompatible (ABO-I) kidney transplantation is now being performed with increasing frequency. For good outcome, most important is to achieve and maintain low anti-blood group antibody titers (ABGATs). Twenty-two patients with ABO-I donors have been studied. The anti-A and anti-B antibody titers (IgG and IgM) were estimated by column agglutination technology using Automated Ortho BioVue System. For desensitization, pretransplant plasmapheresis and/or immunoadsorption and rituximab were used. ABGAT was determined before transplant and periodically after transplant. It was observed that one-third of the patients have low baseline ABGAT. In these cases with low ABGAT, transplant can be performed without any desensitization. In those with titers <1:256, rituximab (two doses of 200 mg weekly) and 3–6 sessions of plasmapheresis can bring down titers to <1:32. In those with titers >1:256, immunoadsorption may be used from the beginning to reduce ABGAT. After transplant, the titers drop to <1:8 in majority. Rise in titers to >1:64 require close observation and biopsy. If there is evidence of antibody-mediated rejection, treatment should be promptly started. Rise in titers 4–6 weeks after transplant is not associated with any graft dysfunction, and hence not of any clinical significance.

AB0-INCOMPATIBLE LIVING DONOR KIDNEY TRANSPLANTATION: THE LONG-TERM OUTCOMES

Background: AB0-incompatible kidney transplantation is one of the ways to effectively expand the pool of living donors. In Russia, this technology has been used for more than 10 years, but until now there have been no reports on its long-term results. Aim: To evaluate the short- and long-term outcomes of the living-related AB0-incompatible kidney transplantations. Materials and methods: We analyzed the results of 49 consecutive AB0- incompatible kidney transplantations, performed from 2011 to 2017. Preoperative management of recipients included administration of rituximab and intravenous immunoglobulin, plasmapheresis and/or selective immunoadsorption, and combination of tacrolimus, mycophenolates and steroids. The target of anti-A/B antibody levels were is 1:8. All patients received standard immunosuppression after transplantation. Results: At baseline, median anti-A/B titer was 1:16 (1:2 to 1:1024) for IgM and 1:4 (0 to 1:512) for IgG. Median rituximab dose was 286 mg/m2 (range, from 94 to 396). To achieve target antibody levels, up to 10 plasmapheresis and/or immunoadsorption sessions (median, 2) were required. There were no deaths during the follow-up. Five grafts were lost, one of them due to hyperacute rejection. The incidence of biopsy-proven rejection was 6%. One-, threeand six-year graft survival was 94%, 90% and 80%, respectively. Conclusion: Kidney transplantation across the AB0-incompatibility barrier is a safe, successful and reasonable option to reduce the organ shortage.

Benefit in long-term response and mortality of treatment with intravenous immunoglobulin prior to plasmapheresis in peripheral polyneuropathies

ObjectivesThe benefits of plasmapheresis (PA) for neurologic autoimmune diseases have been widely demonstrated. Little is known about the long-term neurologic prognosis and course after PA and immunosuppressive (IS) and/or intravenous immunoglobulin (IVIG) treatment. We aimed to analyse features associated with short-term response and long-term outcome and prognosis (neurologic status and mortality) of peripheral polyneuropathy (PP) and central nervous system acute inflammatory disease (CNSAID) treated with PA. Patients and methodsA descriptive, retrospective single-centre study from January 2005 to December 2012. ResultsThere were 26 episodes, which included 16 CNSAID and 10 PP cases. First line therapy included PA (n=4), IS drugs (n=15), and IVIG (n=7). Responses were achieved in 80% and 50% of PP and CNSAID cases, respectively. For PP, first line treatment with IVIG and no IS treatment prior to or during PA were variables associated with short-term response (P=0.067), good or stable neurologic status at the end of follow-up (P=0.008), and lower mortality rate (P=0.008). For CNSAID, initial EDSS score≥7 (P=0.019) was related to long-term good or stable neurologic status. During the study period, 177 sessions were conducted; 3.4% had technical complications and 8.5% clinical complications. However, these incidents were all minor and no PA session had to be discontinued. ConclusionThe response rates achieved in our patients were similar to those of other research. PA has a safe profile but double-blind, controlled studies are needed to evaluate the synergy of sequential treatment with IGIV followed by PA and the possible benefit for long-term outcome.

Desensitization Protocol in Recipients of Deceased Kidney Donor With Donor-Specific Antibody–Low Titers

BackgroundKidney transplantation is the better option for end-stage renal disease (ESRD), but for patients with human leukocyte antigen (HLA) sensitization, the wait times are significantly longer than for patients without antibodies. Many desensitization protocols have been described involving strong immunosuppression, the use of apheresis, and B-cell–modulating therapies. We have designed a desensitization protocol from day 0 for deceased donor kidney transplantation. Our aim was to present our initial experience with five kidney transplant patients. MethodsAll patients had a negative complement-dependent cytotoxicity cross-match. The desensitization protocol included five to seven doses of thymoglobulin (1.25 mg/kg) and three sessions of plasmapheresis (PP) within the first week after transplantation, with intravenous immunoglobulin (500 mg/kg) after each PP session and one dose of rituximab on day 8. The presence of donor-specific antibodies (DSA) was analyzed by use of Luminex technology; levels between 1000 and 3000 mean fluorescence intensity were considered for desensitization. ResultsThe median age was 44 years and median renal replacement therapy time was 9 years. All recipients presented 1 to 3 DSA specificities. There were no severe side effects related to PP, infusion of intravenous immunoglobulin, or rituximab. The median follow-up period was 19.3 months. Median serum creatinine level at last follow-up was 1.7 mg/dL. A kidney biopsy was performed in all patients. Graft and patient survival was 100%. ConclusionsUntil now, few data are available concerning whether HLA-incompatible kidney transplantation after desensitization would benefit patients with ERSD. The desensitization strategy using the combination of PP, low doses of intravenous immunoglobulin, and rituximab at our center resulted in a satisfactory clinical outcome.