Course Of Plasmapheresis Research Articles

Alemtuzumab plus cyclosporine treatment of the autoimmune hemolytic anemia in an adult bowel transplant.

An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft.

Clinical experience of double plasma molecular absorption with a combination of two hemoperfusion machines in treatment of liver failure

To evaluate the effect of the double plasma molecular adsorption system (DPMAS) with a combination of two hemoperfusion machines in treatment of liver failure. A retrospective analysis was conducted. The clinical data from 42 patients with liver failure admitted to Tianjin Second People's Hospital from September 2012 to September 2013 were enrolled. Patients received 166 courses of blood purification treatment, including 97 courses of plasmapheresis (PE) and 69 courses of DPMAS. The DPMAS treatment was performed with a combination of two hemoperfusion machines, with one a blood pump and the other a plasma pump, for 2-3 hours. Liver function, tumor necrosis factor-α (TNF-α), electrolytes and blood routine were determined before and after treatment. Adverse reactions were observed and the nursing experiences were summarized. The survival rate of 42 liver failure patients was 64.29%, and the total bilirubin (TBil), NH3, total bile acid (TBA) and TNF-α were decreased and the albumin (ALB) was increased after PE and DPMAS. Further analysis of the rate of changes after treatment (after treatment/before treatment×100%) showed that when compared with that in DPMAS, the TBil and TBA were decreased significantly in PE [TBil: (62.21 ± 5.51)% vs. (64.39 ± 4.61)%, t=2.683, P=0.008; TBA: (77.10 ± 4.44)% vs. (85.91 ± 6.95)%, t=9.952, P=0.000], and the level of ALB was elevated significantly in PE when compared with that in DPMAS [(113.12 ± 2.90)% vs. (101.87 ± 2.91)%, t=24.602, P=0.000]. NH3 and TNF-α were decreased in both groups withe no statistical significance [NH3: (79.59 ± 5.72)% vs. (80.56 ± 7.56)%, t=0.934, P=0.351; TNF-α: (61.66 ± 4.67)% vs. (62.73 ± 3.67)%, t=1.638, P=0.108]. The blood electrolytes and routine blood test showed that there was no significant change before and after treatments in DPMAS group (K⁺: 3.92 ± 0.83 mmol/L vs. 3.91 ± 0.82 mmol/L, t=0.501, P=0.618; Na⁺: 136.89 ± 5.69 mmol/L vs. 136.74 ± 5.83 mmol/L, t=1.077, P=0.285; Cl⁻: 96.58 ± 3.33 mmol/L vs. 96.55 ± 3.27 mmol/L, t=0.245, P=0.807; white cell count: 5.22 ± 0.93 × 10⁹/L vs. 5.43 ± 1.11 × 10⁹/L, t=1.125, P=0.265; hemoglobin: 110.97 ± 19.20 g/L vs. 112.69 ± 19.67 g/L, t=0.643, P=0.522; platelet count: 105.28 ± 26.82 × 10⁹/L vs. 101.96 ± 3.08 × 10⁹/L, t=0.727, P=0.470). Sixty-four out of the 69 courses of DPMAS treatment were successfully completed, and 5 times were ended because of line coagulation. There was no uncomfortable symptom complained by 53 patients during the treatment. Hypotension occurred for 3 times, and discomfort and nausea during treatment were complained for 9 times. Low fever occurred 4 times after treatment. Those uncomfortable symptoms were relieved spontaneously or by symptomatic treatment. Psychological nursing care should be given to the patients before and after DPMAS to relieve anxiety. Circulating tubes should be well rinsed to prevent line coagulation. General condition of the patient should be closely monitored in order to adjust therapeutic measures. DPMAS could be effectively performed with a combination of two hemoperfusion machines without inadvertent side reactions, and it is safe in operation with smaller amount of plasma.

Anti-glomerular basement membrane disease treated with mycophenolate mofetil, corticosteroids, and plasmapheresis

An 18-year-old woman presented with anemia, pulmonary hemorrhage, and necrotizing glomerulonephritis, and was diagnosed with anti-glomerular basement membrane (anti-GBM) disease. Treatment was undertaken with plasma exchange, mycophenolate mofetil and corticosteroids, due to patient refusal of cyclophosphamide. Clinical remission was successfully induced with this fertility-sparing regimen. A relapse due to therapy non-adherence was successfully treated with a second course of plasmapheresis, mycophenolate, and steroids. Thereafter, 6 months of directly observed therapy resulted in a favorable outcome with well-preserved pulmonary and renal function. This case suggests the possibility that mycophenolate may have a role in the treatment of anti-GBM disease.

Reduced HLA Class II Antibody Response to Proteasome Inhibition

Purpose One major function of the 26S proteasome is antigen processing for HLA class I presentation. Following proteasome inhibition with bortezomib (Bzb), one would expect down regulation of class I expression. We explored the differential effect on donor specific and third-party antibodies in Bzb-treated OHT patients. We postulated that compared to class II responses, Bzb is more effective in preventing de novo antibody production to class I alloantigens. Methods and Materials Pearson correlations among Ab per and across patients were performed for patients receiving Bzb desensitization/AMR therapy from August 2010 to January 2012. Hierarchical clustering was used to find antibody groups of similar response. Treatment consisted of plasmapheresis then Bzb (0.7-1mg/m² IV) on days 1, 4, 7, 10 then a 3-day course of plasmapheresis beginning day 13. Anti-HLA antibody mean fluorescence intensity (MFI) was determined via single antigen Luminex® assay. Results Evaluation of the antibody response patterns to Bzb was done on 9 patients. Out of these, 4 patients shared common antibodies, tested multiple times before and after transplantation. Antibody levels are depicted as a heatmap for the intersection of antibody sets. We found that compared to HLA class I locus A and B, HLA class II antibodies demonstrated a significantly decreased response to Bzb. [ figure 1 ] Conclusions We have identified clusters of antibodies with differing degrees of response to Bzb therapy that could be indicative of a class-specific effect. Class I antibodies show a preferential response to bortezomib, while Class II (specifically HLA-DQ) appeared to respond poorly. Our findings suggests that individualized, HLA-specific therapies targeting specific antibody clusters merits further study.

Survival after Amphotericin B Overdose Treated with Plasmapheresis

To report a case of accidental amphotericin B overdose that was treated with plasmapheresis. A 60-year-old woman with a history of kidney transplant 4 years prior to presentation for a congenital abnormality was admitted for a suspected systemic fungal infection. The patient inadvertently received intravenous amphotericin B deoxycholate 250 mg (4.3 mg/kg) over 2 hours instead of prescribed liposomal amphotericin B. The medication error was discovered 16 hours after administration. She had normal vital signs at that time and reported abdominal pain and general malaise. Results of a metabolic panel were significant for a creatinine level of 2.1 mg/dL and CO₂ of 17 mg/dL. Her serum amphotericin B concentration 33 hours after the initial dose was 4.9 μg/mL. She subsequently received 5 courses of plasmapheresis and 3 courses of hemodialysis and ultimately did not develop any further renal injury, as well as hemolysis, cardiovascular collapse, dysrhythmias, or severe electrolyte abnormalities. The dosing differences between nonliposomal and liposomal preparations of amphotericin B can be as high as 50-fold. Reported adverse events from overdose in both animal models and human case reports include renal insufficiency, hemolysis, thrombocytopenia, electrolyte abnormality, and cardiac dysrhythmias. There have been previous reports of similar errors that have led to death. Furthermore, amphotericin B has been shown to be poorly dialyzable. Our patient's serum amphotericin B concentration decreased after she received plasmapheresis, and she did not develop severe complications. We describe a patient who survived a 4-fold overdose of amphotericin B because of a medication error. The use of plasmapheresis may have enhanced the elimination of amphotericin B and may have contributed to the positive outcome. However, the role of plasmapheresis in amphotericin overdose is not fully understood.

Brachial amyotrophic diparesis associated with anti-Hu positive anterior horn cell disease and autonomic disorder

A 32 year old, right-handed woman was admitted to our hospital with flaccid, atrophic and proximally accentuated paresis of both arms, which had been slowly progressive over the previous five months. At the time of admission to our hospital she was unable to lift her arms or to feed herself. The clinical examination revealed tonic pupils, purely motor symptoms with symmetric atrophy of shoulder, arm and hand muscles and a high-grade flaccid paresis of both arms (proximal MRC: 0–1; distal MRC: 2–3/5). The strength of the legs was normal. The sensory system was not affected. While the upper limbs showed areflexia, tendon reflexes of the legs were symmetrically brisk, yet pyramidal signs were not detectable. Altogether, the clinical syndrome presented by the patient was compatible with a ‘‘flail arm syndrome’’ [1, 2]. Nerve conduction studies were compatible with anterior horn cell affection, and EMG recordings documented widespread denervation of the arm muscles. Conduction of motor nerves of the legs and sensory nerves of the arms and legs were not affected. Likewise, SSEPs were normal and MEPs showed no upper motor neuron involvement. Further investigations revealed sinus tachycardia, reduced heart rate variability, reduced sympathetic skin reaction and hypomobility of the oesophagus as signs of autonomic dysfunction. Interestingly, the MRI showed bilateral T2 hyperintensities in the area of the anterior column of the entire spinal cord without gadolinium enhancement. The alterations were most pronounced in the cervical medulla (Fig. 1, 2). Poliomyelitis could be excluded since the patient had been vaccinated 5 years earlier. In the serum and in the cerebrospinal fluid, high titers of anti-Hu antibodies (1:3200 in the serum) were detected by IgG-immunoblot and indirect immunofluorescence. Testing for various other anti-neuronal antibodies (including anti-Yo,-Ri,-Ma1/2, -CV2,-Amphiphysin,-NMDAR,-AMPA,-VGKC,-GLU1,GLU2,-Tr,-Aquaporin-4,-SOX1,-Myelin,-Glycin,-GAD,MAG) was negative. Isoelectric focussing detected oligoclonal immunoglobulins, which in part were exclusively present in the CSF, others were identical in CSF and in serum. CSF concentrations of protein, lactate and glucose, as well as cell count (4 leukocytes/ll), were normal. Tumor markers Cyffra 21–1 (4.2 ng/ml, normal range\3.3 ng/ml) and Chromogranin (209 lg/l, normal range 19–98 lg/l) were somewhat elevated in the serum, suggesting a paraneoplastic syndrome as underlying cause of the neurological symptoms. In spite of extensive efforts including CT-scans of thorax and abdomen, whole body FDG oncology-PET, endoscopy of the entire gastrointestinal tract, bronchoscopy, gynaecologic examination and DOTATOC PET–CT, no neoplasm was detected. Clinically, the neurological symptoms did not respond to two 5-day courses of high-dose i.v. methylprednisolone, several courses of plasmapheresis as well as infusions of IVIG and cyclophosphamide. Instead, the condition further deteriorated. She developed a neurogenic weakness of the breathing muscles, which necessitated mechanical ventilation and J.-I. Lee (&) P. Albrecht H.-P. Hartung N. Goebels Department of Neurology, Medical Faculty, Heinrich-Heine University, Moorenstr. 5, 40225 Duesseldorf, Germany e-mail: John-Ih.Lee@uni-duesseldorf.de

Guillain-Barré-Syndrom mit Dysphagie nach Sinusitis frontalis

One week after an acute sinusitis, a male patient developed a hypernasal voice, dysphagia, diplopic images, ataxia and paresthesias. He had paresis of the glossopharyngeal and abducens nerves, weakness of the arms and legs, and reflex deficiency. The neurography showed a motor axonal demyelinating neuropathy, so that the diagnosis of Guillain-Barré syndrome was made. After five courses of plasmapheresis, the symptoms improved rapidly.

Plasmapheresis to Treat Hypertriglyceridemia in a Child With Diabetic Ketoacidosis and Pancreatitis

A 10-year-old girl presented with diabetic ketoacidosis, shock, and severe abdominal pain. She was found to have acute pancreatitis and acute kidney injury after shock resuscitation and severe persistent hypertriglyceridemia. The severe hypertriglyceridemia was treated with 1 course of plasmapheresis, which corrected the triglyceride level and was temporally associated with improvement of the abdominal pain and renal dysfunction. Diabetes is known to contribute to an elevated triglyceride level, especially in the setting of an underlying lipid disorder. However, no such disorders were found in this patient. To the best of our knowledge, this is the first report of a pediatric patient presenting with the triad of severe hypertriglyceridemia, diabetic ketoacidosis, and pancreatitis treated successfully with plasmapheresis.

Bortezomib-based chemotherapy for light chain deposition disease presenting as acute renal failure

We report a case of kappa light chain deposition disease (LCDD) associated with multiple myeloma in a patient presenting with acute renal failure, 2+ proteinuria and hypercalcemia. Serum protein electrophoresis showed an M-spike at 0.1 g/dL. 24-h urine protein electrophoresis showed Bence-Jones proteinuria of 3.8 g. Serum-free light chain assay found excess kappa chains at 3080 mg/L, with normal lambda and an elevated kappa:lambda ratio of 124.7. A kidney biopsy revealed kappa light chain nephropathy with PAS-negative tubular casts in the cortex and outer medulla. Bone marrow biopsy showed 15% kappa-restricted plasma cells. Serum beta-2 microglobulin level was elevated at 7.94 mg/dL. The patient received a 3-day course of plasmapheresis followed by eight cycles of bortezomib (Velcade®), liposomal doxorubicin, and dexamethasone (VDD) and did not require hemodialysis. As partial response was not achieved, treatment was continued with three cycles of bortezomib, cyclophosphamide, dexamethasone, and thalidomide, followed by thalidomide maintenance at 100 mg daily. Thirty-two months after the diagnosis, the patient's renal function was improved and he achieved a partial response. This case underlines the feasibility and effectiveness of bortezomib-based chemotherapy in the treatment of LCDD with severe renal dysfunction.

Alleviation of exogenous insulin requirement in type 1 diabetes mellitus after immunoablation and transplantation of autologous hematopoietic stem cells

An essential component of type 1 diabetes mellitus is autoaggression of the immune system against insulin-secreting pancreatic cells. It is thought that early destruction of the autoaggressive mechanism prior to the complete damage of beta cells should halt this process. In a 28-year-old male patient with a 4-week history of type 1 diabetes mellitus, three courses of plasmapheresis had been performed before cyclophosphamide, 2 g/m2 body surface area, was administered and hematopoietic cells were obtained. Six weeks after the diagnosis, 4 doses of cyclophosphamide 50 mg/kg body weight were again administered together with antithymocyte globulin, and autologous hematopoietic cells were transplanted. The procedure was associated with no significant side effects. Insulin requirement started to drop from the first course of plasmapheresis, and the patient has remained normoglycemic with no need of exogenous insulin or other hypoglycemic agents since the third week after the procedure, which has been 5 months until publication of this report. Independence from exogenous insulin is associated with the implemented therapy (a gradual decrease in insulin requirement has been observed after consecutive stages of the immunosuppressive treatment, with total discontinuation after bone marrow transplantation). The course of the disease and the type of treatment may suggest that such medical procedures could eliminate autoaggressive mechanism in diabetes and prevent further degeneration of insulin-producing cells, thus becoming a new therapeutic option for patients with type 1 diabetes mellitus.

Efficay of plasmapheresis in a case of severe pulmonary hemorrhage in pediatric Systemic Lupus Erythematosus

We report the case of a 13 year old girl with SLE initially treated with hydroxycloroquine for a mainly mucocutaneous involvement. Four months from onset, she presented renal involvement and an acute hemoptysis and she was admitted to our pediatric department. She also presented fever and abnormal laboratory findings (high serum creatinine level, severe proteinuria, Hb of 6.4 g/dl, high titre specific autoantibodies, low C3 and C4 levels). Kidney biopsy underlined stage 4a lupic nephropathy; chest X-ray and CT scan showed diffused alveolar involvement, as for pulmonary hemorrhage. Cerebral SPECT showed abnormalities consistent with neurolupus. The patient underwent blood transfusion, and was treated with high doses of metilprednidsolone plus cyclophosphamide. Due to refractory anemia and worsening of respiratory symptoms (dispnoea with low peripheral oxygen saturation), she was additionally treated with 3 courses of Rituximab, and two further blood transfusions, with clinical stabilization but no major amelioration. After one week, severe clinical worsening with important dyspnoea, cutaneous pallor, poor general condition, low peripheral oxygen saturation (78%) was present: Hb level was 4 g/dl, chest X-ray showed worsened alveolar involvement. The patient was treated with 3 courses of plasmapheresis and two blood transfusions, with immediate response of the respiratory symptoms, recovery from anemia and marked improvement of the chest X-ray. Pulmonary hemorrhage is a severe complication in SLE associated with high mortality. It is described in 5–6% of patients, presenting with anemia, hemoptysis, respiratory symptoms and characteristic changes in chest imaging. We suggest plasmapheresis as an effective treatment to be considered promptly when suspecting this life-threatening complication.

Clinical Characteristics of Patients with a RBC Fragment Flag on the Advia® 2120 Automated Hematology Analyzer.

The clinical diagnosis of thrombotic thrombocytopenia purpura (TTP) is a difficult one to make. It is based on clinical criteria, one of which is a microangiopathic hemolytic anemia, characterized morphologically by the presence of schistocytes on the peripheral blood smear. The ADVIA 2120 automated hematology analyzer is able to quantify the presence of red blood cell (RBC) fragments. We studied the ability of the ADVIA 2120 to be able to detect RBC fragments in the blood of TTP patients, and the characteristics of all patients in whom RBC fragments were obtained. During the study period, 6 TTP patients were studied. The initial numbers of RBC fragments ranged from 0.02–0.05 × 106 cells/μl. During the course of plasmapheresis, these numbers decreased to 0.00–0.02 × 106 cells/μl, corresponding to a rise in the platelet count. Figure Figure In the course of a month, 52 blood samples on 39 patients were flagged by the hematology analyzer to have RBC fragments (0.01–0.12 × 106 cells/μl). 52 Samples with RBC Fragment Flag Hemoglobin Platelets RDW Range 4– 14.3 g/dl 5–906 × 103/ul 13.9– 28.6% Number Abnormal 46 (<13.0 g/dl) 23 (<160 × 103/ul) 51 (>14.1%) Within this population, there were two patients with TTP, and one with DIC. Four of the samples did not have detectable schistocytes upon visual inspection of the peripheral blood smear. There were 19 samples from 14 patients who had RBC fragment counts ≥ 0.04 × 106 cells/μl. 19 Specimens with RBC Fragments ≥ 0.04 × 106/ul Hemoglobin Platelets RDW Range 8– 14.1 g/dl 59– 906 × 103/ul 16.4– 25.3% Number Abnormal 15 (<13 g/dl) 4 (<160 × 103/ul) 19 (>14.1%) The diagnoses in these 14 patients were iron deficiency anemia (4 patients), thalassemia trait (2), acute lymphoblastic leukemia (2), and one each with TTP, sickle cell anemia, heart failure, kidney stone, cerebrovascular accident (CVA), and end stage renal disease. We conclude that the RBC fragment flag on the ADVIA 2120 is nonspecific. Although it does detect schistocytes in TTP, these are often present in low numbers. Quantitatively, the most numerous RBC fragments are found in diseases with marked anisopoikilocytosis, such as iron deficiency anemia.

Polyclonal Antibody-Induced Serum Sickness Presenting as Rapidly Progressive Descending Paralysis

Polyclonal antibody-induced serum sickness is an immune-complex mediated illness that is commonly manifested as fever, arthralgias, arthritis, lymphadenopathy, with or without renal failure. To our knowledge, an associated rapidly progressive descending paralysis has never been documented. We report a case of a living related renal transplant recipient who presented with serum sickness 1 week after the completion of a 7-day course of rabbit antithymocyte globulin (thymoglobulin at a dose of 1.5 mg/kg/day) for presumed severe acute cellular rejection due to inadvertent discontinuation of tacrolimus immunosuppression. As the patient was taking daily aspirin at the time, a biopsy was not performed. On exam, she was found to have a swollen and tender graft. Her serum creatinine peaked at 2.9 mg/dl and returned to her baseline at 1.3 mg/dl after thymoglobulin therapy. At one week after discharge, however, the patient presented with polyarticular arthralgias, myalgias, back pain, trismus, severe muscle weakness and descending paralysis that progressed within a matter of hours. She also developed a generalized pruritic skin rash that resolved with self-medicated diphenhydramine. On exam, she was noted to have severe joint tenderness at the knees, ankles, shoulders, wrists, and mandible. Neurological assessment revealed bilateral proximal greater than distal weakness, hyperreflexia, and ankle clonus. A clinical diagnosis of thymoglobulin-induced serum sickness was entertained although the neurological findings also raised the possibility of central nervous system pathology, and/or inflammatory myopathies. Chemistry panel revealed a slight increase in serum creatinine to 1.5 mg/dl. Due to her debilitating symptoms, therapeutic plasmapheresis (TPE) was initiated the day of admission pending further evaluation with brain magnetic resonance imaging, cervical spine, and appropriate serologic testing. The patient had immediate relief of her joint pain during plasmapheresis, and more interestingly, drastic improvement of her extremity weakness by the end of the session. She received a second course of plasmapheresis on hospital day 2 due to residual extremity “discomfort” and had complete resolution of her symptoms. Her serum creatinine decreased to 1.1 mg/dl. At 1 week follow-up, the patient reported doing well and had returned to her routine daily activities. The use of TPE in the treatment of serum sickness after antithymocyte globulin therapy has been well reported (1–3). Compared to pulse steroid or analgesics, TPE accelerates the time to recovery (10–14 days vs. 24–48 hr, respectively) (1–3). To our knowledge, this is the first case report of thymoglobulin-induced serum sickness presenting as descending paralysis. Phuong-Thu T. Pham Division of Nephrology, Kidney, and Pancreas Transplantation David Geffen School of Medicine at UCLA Los Angeles, CA Phuong-Mai Pham Department of Medicine Central Maine Medical Center Lewiston, ME Jeffrey M. Miller Division of Hematology/Oncology Olive View UCLA Medical Center Sylmar, CA Phuong-Chi T. Pham Division of Nephrology, Olive View UCLA Medical Center Sylmar, CA

Patient with antibody-negative relapse of Goodpasture syndrome

Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of hemodialysis treatment. It is important to consider that in patients with anti-GBM disease antibody-negative relapses are possible.

Treatment of antibody-mediated rejection with high-dose immunoglobulins in ABO-incompatible liver transplant recipient

ABO-incompatible liver transplantation (LT) entails high risk of antibody-mediated rejection (AMR) and poor graft survival. Different treatment modalities have been reported, but none with use of a 2-week course of high-dose polyclonal i.v. immunoglobulins (IVIg) associated with plasmapheresis without the use of steroid pulses or monoclonal antibody. A 60-year-old male patient with blood-group O, Caucasian, underwent urgent LT for acute liver failure after hepatectomy for HCV-related hepatocellular carcinoma. He was grafted with a 66-year-old, blood-group A, HCV-positive liver graft. Pretransplant conditioning consisted of plasmapheresis and immunosuppression was triple with tacrolimus (TAC), steroids, and mycophenolate mofetil with anti-IL2-R monoclonal antibodies, plasmapheresis if hemagglutinin level >1:8, and extracorporeal photopheresis. After reduction of liver function tests to baseline, the patient presented a tenfold increase in alanine aminotransferases (ALT) levels 7 days post-transplantation. AMR was confirmed on histology. Treatment consisted of IVIg (1.5 g/Kg/daily for the first 7 days, and 1 g/Kg/daily from day 8 to 14) with a 14-day course of plasmapheresis. No side effect was observed and daily blood IgG levels ranged between 24.4 and 36.4 g/l. At the end of the scheduled course ALT returned to baseline. A control liver biopsy 55 days after LT showed no rejection and replacement of necrosis with fibrous strands. This case may support the role of high-dose IVIg for treatment and/or prophylaxis of severe AMR.

Early recurrence of primary focal segmental glomerulosclerosis in an older cadaveric renal allograft recipient resistant to plasmapheresis

We report an unusual case of recurrent primary focal segmental glomerulosclerosis in an apparent low-risk cadaveric renal allograft recipient only 2 days after transplantation, who did not respond to repeated courses of plasmapheresis.

Heparin-induced thrombocytopenia type II: A rare but significant complication of plasma exchange

Type II heparin-induced thrombocytopenia (HIT) is a rare but well-recognised and potentially life-threatening complication of unfractionated heparin therapy, and has been reported in association with heparin locks for central venous lines. We report a case of type II HIT complicated by iliofemoral deep venous thrombosis and pulmonary embolism in a 43-year-old woman in the course of plasma exchange for myasthenia gravis. A Gamcath central venous line had been inserted femorally due to poor peripheral venous access, and this was locked with heparin 5000 U/ml between procedures. Twelve days after initial heparin exposure, she presented with new-onset thrombocytopenia, a painfully swollen right leg, and pleuritic pain. Deep venous thrombosis and pulmonary embolism were confirmed radiologically, and serology for heparin/PF4 antibodies was unequivocally positive. The line was removed, and she was successfully managed with intravenous lepirudin, switching to warfarin on platelet recovery. This case demonstrates that Type II HIT can occur in association with heparin line locks in the course of plasmapheresis, despite previous reports of successful use of plasma exchange to treat Type II HIT.

Complications of double‐filtration plasmapheresis

Double-filtration plasmapheresis (DFP) removes high-molecular-weight molecules semiselectively, thereby minimizing albumin loss, but with subsequent need for fluid substitution. The purpose of this study was to establish the overall incidence of complications during DFP and to analyze the possible contributory factors. From November 1993 to October 2003, a total of 2502 plasmapheresis procedures were performed during 515 courses of plasmapheresis in 335 patients. The medical and technical records for these patients were reviewed. The overall frequency of complications was 67.5 percent per patient, 60.0 percent per course, and 26.3 percent per procedure. The rate of major complications was 18.2 percent of patients, 13.2 percent of courses, and 2.7 percent of procedures. Hemolysis, the most common complication of DFP, occurred in 20 percent of patients. A total of 83 (3.3%) episodes of hypotension were documented in 63 patients. Vascular-access-related complications constituted approximately 17 percent of the total. There was one documented episode of clinically overt bleeding from laboratory coagulopathy necessitating infusion of fresh plasma to stop bleeding. There was no allergic reaction or mortality associated with the DFP procedures. The per-patient frequency of hypotension was higher in cases of chronic inflammatory neuropathy (35.1%) compared to the other illness categories (15.4%-17.3%), with marginal significance demonstrated (p = 0.0611). The safety of DFP treatment has been confirmed by this study. High-risk patients should be monitored carefully during treatment to minimize the occurrence of complications.

Successful treatment of extremely severe fetal anemia due to Kell alloimmunization

BACKGROUND: Repeated plasmapheresis was used to prevent fetal death from severe anti-Kell alloimmunization until intrauterine transfusions were feasible. CASE: Repeated maternal plasma exchanges ( N = 40) beginning at 7 weeks’ gestation were used to treat severe anti-Kell alloimmunization. Ultrasound examination at 19 weeks’ gestation revealed diffuse hydrops in this fetus (umbilical venous hemoglobin, 1.2 g/dL), which then required nine intrauterine transfusions through 34 weeks. A healthy 3840-g girl was delivered by cesarean delivery at 36 weeks. Despite aplastic anemia during the first 3 months of life, she is healthy and has no observable abnormalities at age 8. CONCLUSION: A highly aggressive course of plasmapheresis and intrauterine transfusions can successfully treat fetal anemia caused by anti-Kell alloimmunization even when fetal hemoglobin is extremely low.

Successful Treatment of Extremely Severe Fetal Anemia Due to Kell Alloimmunization

In Brief BACKGROUND Repeated plasmapheresis was used to prevent fetal death from severe anti-Kell alloimmunization until intrauterine transfusions were feasible. CASE Repeated maternal plasma exchanges (N = 40) beginning at 7 weeks' gestation were used to treat severe anti-Kell alloimmunization. Ultrasound examination at 19 weeks' gestation revealed diffuse hydrops in this fetus (umbilical venous hemoglobin, 1.2 g/dL), which then required nine intrauterine transfusions through 34 weeks. A healthy 3840-g girl was delivered by cesarean delivery at 36 weeks. Despite aplastic anemia during the first 3 months of life, she is healthy and has no observable abnormalities at age 8. CONCLUSION A highly aggressive course of plasmapheresis and intrauterine transfusions can successfully treat fetal anemia caused by anti-Kell alloimmunization even when fetal hemoglobin is extremely low. Extremely severe Kell alloimmunization was successfully treated with plasmapheresis and intrauterine transfusions.