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PubMedDate not supplied

Reversible severe vascular rejection after plasmapheresis.

Vascular rejection in a transplanted child is frequently associated with bad prognosis on the short and long term. All therapeutic modalities available nowadays as monoclonal antibodies, intravenous cyclosporine or plasma exchange have given conflicting results. We report the case of a boy who was transplanted from his mother at the age of three. His primary disease was a cortico-resistant rapidly progressive glomerulonephritis; the immediate postoperative period was satisfactory but he developed a severe vascular rejection on day 5 post-transplantation, while on triple immunosuppression therapy associating antithymocyte globuline, prednisolone and azathioprine. Acute vascular rejection was corticoresistant but responded well to plasma exchange. In our patient, we believe that plasmapheresis was efficaceous on the reversibility of vascular rejection and moreover that he had not responded to corticotherapy even a week post methylprednisolone in the absence of any change in immunosuppression during plasmapheresis. No randomized study was realized until now to prove the efficacy of plasmapheresis in children with vascular rejection. The sporadic cases reported on the reversibility of such rejection after plasmapheresis, the innocuity of this therapy, if well performed in children, encourage us to consider this treatment modality in resistant vascular rejection.

Acute Disease↗

PubMed1981

Plasmapheresis in refractory generalized myasthenia gravis.

A group of 16 patients with severe generalized myasthenia gravis (MG) (five with thymoma) that was resistant to anticholinesterases, thymectomy, and corticosteroids were treated by plasmapheresis. Twelve patients showed an excellent clinical response. Plasmapheresis is an effective treatment modality for many patients with severe generalized MG resistant to other forms of therapy. Unfortunately, the beneficial results are only transient and periodic plasmapheresis treatments are necessary.

Adult↗

PubMed1998

Thrombotic thrombocytopenic purpura associated with HIV and visceral Kaposi's sarcoma treated with plasmapheresis and chemotherapy.

We present a case of a patient who is HIV positive and developed both thrombotic thrombocytopenia purpura and visceral Kaposi's sarcoma (KS) with hemorrhage. This case presents a difficult management problem in that the patient's bleeding originated from KS lesions and did not quickly abate with plasmapheresis therapy despite both clinical and laboratory improvement after 2-4 days. Chemotherapy was initiated on day 13 and the patient's condition improved markedly afterward. We believe the addition of chemotherapy to plasmapheresis hastened the improvement of our patient's thrombotic thrombocytopenic purpura (TTP) and KS-related bleeding. Therefore, under similar conditions, we recommend combining plasmapheresis and chemotherapy at the onset of therapy.

Adult↗

PubMed1997

Cholesterol biosynthesis in normocholesterolemic patients after cholesterol removal by plasmapheresis.

Plasmapheresis and low-density lipoprotein (LDL)-apheresis are recognized procedures for the treatment of hyperlipidemia resistant to diet and lipid-lowering drugs and provide information on cholesterol synthesis in hypercholesterolemic patients. However, cholesterol synthesis after acute cholesterol removal from plasma has never been investigated in normocholesterolemic patients. In this study, cholesterol synthesis was evaluated in three normocholesterolemic patients by determination of plasma lathosterol, lathosterol-to-cholesterol ratio, and plasma mevalonic acid. In a short-term kinetic study, samples were collected before and after plasmapheresis and every 6 hours during 24 hours. In the second part of the study, cholesterol synthesis was evaluated daily for 3 days. In normocholesterolemic patients, cholesterol returns to basal levels in 3 days. However, cholesterol removal did not result in a significant increase in lathosterol-to-cholesterol ratio or in plasma mevalonic acid, despite a slight increase in lathosterol. In contrast, when repeated plasma exchanges induced a dramatic hypocholesterolemia (< 1 mmol/liter), an acute but transient stimulation of cholesterol synthesis was observed (lathosterol/cholesterol ratio and MVA, respectively, increase from 8.2 to 22.3 and from 28 nmol/liter to 98 nmol/liter). This study shows that cholesterol synthesis is not stimulated by plasmapheresis in normocholesterolemic patients but is enhanced in dramatic hypocholesterolemic patients (< 1 mmol/liter).

Cholesterol↗

PubMed1998

Treatment of congenital afibrinogenemia with cryoprecipitate collected through a plasmapheresis program using dedicated donors.

A child with afibrinogenemia was evaluated for prophylactic cryoprecipitate transfusion due to recurrent episodes of traumatic bleeding. The parents would only consider ongoing transfusion therapy if a limited donor program could be established. Automated plasmapheresis was performed on a regular basis on the patient's parents and a limited number of selected donors. Studies on the initial units collected demonstrated that a single 500 mL plasmapheresis yielded a mean of 606 mg fibrinogen in the cryoprecipitate, which was stored in two bags. A total of 166 U of cryoprecipitate from 84 individual plasmapheresis donations were transfused prophylactically every 2-3 weeks over a 16-month period. Compared to transfusions from random donors, the use of a limited number of apheresis donors resulted in a donor exposure reduction of 87%. Clinically, the patient has had minimal bleeding during this period.

Afibrinogenemia↗

PubMed1999

Plasmapheresis for crescentic IgA nephropathy: a report of two cases and review of the literature.

Idiopathic IgA nephropathy is widely regarded as a slowly progressive disease that not infrequently results in end-stage renal failure. Only a minority of patients present with either a rapidly progressive form of glomerulonephritis, or with end-stage renal failure. Anecdotal reports of improved renal function after treatment with plasmapheresis have been published, but the efficacy of this therapy remains controversial. We describe the course of two young males presenting with uremia, hypertension, nephrotic-range proteinuria, and crescentic glomerulonephritis on renal biopsy. Both patients underwent therapy with steroids, immunosuppressive agents, and plasmapheresis without an appreciable improvement in renal function. A review of the literature does not offer any conclusive data to support the role of plasmapheresis in the treatment of rapidly progressive glomerulonephritis due to IgA nephropathy and points out the need to define criteria that may identify subsets of patients with this disorder who may potentially benefit from plasma exchange therapy. J. Clin. Apheresis 14:185-187, 1999. Published 1999 Wiley-Liss, Inc.

Adolescent↗

PubMed1983

Plasmapheresis in a patient with angioimmunoblastic lymphadenopathy. Improvement in clinical and immunologic abnormalities.

A patient with steroid resistant, allergen related angioimmunoblastic lymphadenopathy underwent a course of six plasmaphereses during a three-week period. A 75% reduction in lymph node size along with the disappearance of her night sweats occurred. Immunologic abnormalities prior to plasmapheresis included the presence of elevated levels of circulating immune complexes, high levels of spontaneous mononuclear cell blastogenesis and abnormal mitogen responses to Conconavalin A and phytohemagglutinin. Following plasmapheresis there was a marked reduction in immune complex levels, and return of spontaneous blastogenesis and mitogen responses to normal levels. Mechanisms for the beneficial effect seen in this patient include removal of: (1) the antigenic stimulus; (2) antigen antibody complexes; and (3) other humoral factors which may modulate lymphocyte or macrophage function. Additional studies of plasmapheresis are warranted in selected patients with allergen related AIL.

Aged↗

PubMed1982

Plasmapheresis and immunosuppressive drug therapy in the Eaton-Lambert syndrome.

Five patients with Eaton-Lambert syndrome (ELS) without associated malignancy were first treated by plasmapheresis alone, and subsequently by plasmapheresis combined with prednisone and azathioprine therapy. Three of the five were also treated by the immunosuppressive drugs alone. Although all three therapeutic regimens resulted in some clinical and electromyographic (EMG) improvement, the greatest improvement was seen during plasmapheresis combined with prednisone and azathioprine. The initial evoked compound muscle action potential (CMAP) increased during treatment in all patients, while facilitation of the initial CMAP by tetanic stimulation decreased. Overall, the EMG decrement elicited at 2 Hz lessened in four of the five patients. Serious complications probably attributable to guanidine toxicity developed in three patients: two had renal failure and one had gastrointestinal and urinary tract bleeding. Our results suggest that immunosuppressive therapy may have a place in the management of ELS and that circulating factors such as autoantibody may participate in its pathogenesis.

Action Potentials↗

PubMed1981

Plasmapheresis in systemic lupus erythematosus: a cautionary note.

Plasmapheresis was used as the sole treatment modality in 4 patients with systemic lupus erythematosus. The presence of circulating immune complexes in this group of patients was not predictive of response to plasmapheresis. Lymphocytotoxic antibody levels fell in 3 of the 4 patients, and no consistent changes in T lymphocytes, T lymphocyte subsets, complement, or immunoglobulin levels were observed. Followup during the subsequent 6 months showed deterioration in clinical and laboratory parameters in all 4 patients, and the protocol was discontinued. Plasmapheresis without concomitant therapy may be detrimental to certain patients with systemic lupus erythematosus.

Adult↗

PubMed2003

Plasmapheresis for the treatment of acute pancreatitis induced by hemophagocytic syndrome related to hypertriglyceridemia.

Histiocytic cytophagic panniculitis is an unusual form of hemophagocytic syndrome related to T-cell lymphoma that can be responsible for hypertriglyceridemia. The elevation of serum triglycerides, usually in the setting of familial lipidemia or during uncontrolled diabetes mellitus, is a well-recognized cause of pancreatitis. We report here on the treatment by plasmapheresis of a patient suffering from a T-cell lymphoma-related panniculitis who presented with an acute pancreatitis induced by hypertriglyceridemia. The Ranson scoring system at day 2 was increased and predicted a poor course while clinical features worsened. The patient was treated with one plasmapheresis that allowed a dramatic (89%) decrease in the triglycerides level. The acute pancreatitis resolved and the patient was discharged from the intensive care unit at day 5 with lipids and pancreatic enzyme levels within normal range. To our knowledge, this is the first case of acute pancreatitis induced by hypertriglyceridemia in the setting of an uncontrolled cytophagic histiocytic panniculitis successfully treated by plasmapheresis.

Acute Disease↗

PubMed2003

Plasmapheresis as rescue therapy in accelerated acute humoral rejection.

Accelerated acute humoral rejection (AHR) continues to occur in renal transplantation despite improved crossmatching, with potentially devastating consequences. Between 1 June 1998 and 31 December 2000, 440 renal transplants were performed in our center. AHR was diagnosed by the demonstration of typical pathological features on renal histology and positive direct immunofluorescence or detection of anti-HLA antibodies in serum. AHR developed in 20 (4.5%) of our renal transplant recipients, nine male and eleven female at an average of 16.3 days post transplantation. All of these patients had a negative current cytotoxic crossmatch prior to transplantation. The median serum creatinine at diagnosis was 5.96 mg/dL, and 83% of these individuals developed oliguric renal failure requiring dialysis after having initially attained good graft function (median of best serum creatinine before AHR was 2.64 mg/dL). The 18 recipients who had not infarcted their grafts at the time of diagnosis of AHR received plasmapheresis in conjunction with intensification of their immunosuppressive regimen. This regimen was successful in reversing AHR in 78% of those treated with apheresis. In the 14 responders, graft survival at 6 months was 100% and at 12 months was 91%. Median serum creatinine at 6 and 12 months was 1.26 and 1.33 mg/dL, respectively. Patients received an average of 8.1 plasma exchanges. However, responders received a significantly higher frequency of plasmapheresis (P =.0053), despite undergoing a similar number of exchanges overall. Plasmapheresis appears to be an effective modality for reversing AHR and maintaining graft function.

Adult↗

PubMed2001

Unintentional platelet removal by plasmapheresis.

Therapeutic plasmapheresis may remove platelets as well as plasma. Unintentional platelet loss, if not recognized, may lead to inappropriate patient assessment and treatment. A patient with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is reported in whom persistent thrombocytopenia was interpreted as continuing active disease; thrombocytopenia resolved only after plasma exchange treatments were stopped. This observation prompted a systematic study of platelet loss with plasmapheresis. Data are reported on platelet loss during 432 apheresis procedures in 71 patients with six disease categories using three different instruments. Comparing the first procedure recorded for each patient, there was a significant difference among instrument types (P<0.001); platelet loss was greater with the Fresenius AS 104 (17.5%, N = 21) than with the COBE Spectra (1.6%, N = 26) or the Haemonetics LN9000 (2.6%, N = 24). With all procedures, platelet loss ranged from 0 to 71%. Among disease categories, platelet loss was greater in patients with dysproteinemias who were treated for hyperviscosity symptoms. Absolute platelet loss with the first recorded apheresis procedure, in the 34 patients who had a normal platelet count before the procedure, was also greater with the AS 104 (2.23 x 10(11) platelets) than with the Spectra (0.29 x 10(11) platelets) or the LN9000 (0.37 x 10(11) platelets). In 39 patients in whom data were collected on consecutive days, platelet removal by plasmapheresis correlated with a decreased patient platelet count (r = 0.40, P = 0.011). In these 39 patients, the platelet counts were significantly decreased at 24 hours (P = 0.002).

Amnesia↗

PubMed2001

Hemodynamic effects of the different vascular accesses used for double-filtration plasmapheresis.

Systematic investigations of hemodynamic status during double filtration plasmapheresis (DFP) are rare in the literature. To investigate the hemodynamic effects of the vascular access chosen for DFP, variations in blood pressure (BP) and pulse rate (PR) induced acutely by DFP were prospectively analyzed in 46 myasthenia gravis (MG) patients a standard DFP protocol with isovolumetric saline fluid replacement. BP and PR were monitored at 30-min intervals (baseline, M30, M60, M90, and M120) during the procedures. The patients were randomized into central vein (CV) and peripheral vein (PV) groups based on the vascular access used. Systolic BP (SBP) dropped significantly at M60 (P < 0.05), M90 (P < 0.001), and M120 (P < 0.001) when compared to the baseline level. Symptomatic hypotension was not observed in any of the 46 sessions. SBP values during DFP in the CV group were significantly lower than the PV group's at M60 (93.1 vs. 101.0%, P < 0.05) and marginally lower at M90 (91.2 vs. 97.2%, P = 0.06). There was no significant difference in diastolic BP changes between the two groups. In the CV and PV groups, PR changes during plasmapheresis also differed at M90 (103.4 vs. 94.5%, P < 0.001) and M120 (101.3 vs. 95.0%, P < 0.05). The significantly lower SBP during DFP in the CV group at M60 may be due to the high central vein flow rate and resultant delay in volume replacement. In conclusion, the vascular access selected for DFP plays a role in the pathogenesis of plasmapheresis-related hypotension. Controlling flow rates may help to prevent hypotension.

Adolescent↗

PubMed2004

Plasmapheresis in acute disseminated encephalomyelitis.

High-dose corticosteroid administration has been regarded as the main therapy for acute disseminated encephalomyelitis (ADEM). However, some patients with ADEM do not respond well to this treatment. We successfully used plasmapheresis to treat 2 patients who had ADEM. We also compared our patients' symptoms and clinical outcomes to those from previous reports. Plasmapheresis may be indicated not only for severe cases that fail to improve after high-dose corticosteroid treatment but also for first-line treatment. Additional large, controlled, double-blinded trials are needed to clarify the role of plasmapheresis in ADEM.

Adrenal Cortex Hormones↗

PubMed1982

Use of lymphoplasmapheresis or plasmapheresis in the management of acute renal allograft rejection.

Several recent reports have documented the value of intensive plasmapheresis as an adjunct to standard immunosuppressive therapy for patients suffering acute renal allograft rejection. We have treated four rejection episodes in three patients with intensive plasmapheresis and two rejection episodes in two additional patients with intensive lymphoplasmapheresis. Five of six rejection episodes were reversed, and four of the five patients treated have retained functioning grafts for follow-up periods ranging from 4 months to 3 years. Previous investigators have reported encouraging results using plasmapheresis, and we believe our experience supports the requirement for further controlled studies with this procedure. Moreover, we note that no previous work has been described with lymphoplasmapheresis and suggest that removal of lymphocytes, in addition to plasma, may further augment immunosuppression in the treatment of renal allograft rejection.

Acute Disease↗

PubMed1983

The effect of plasmapheresis and drug immunosuppression on T-cell subsets as defined by monoclonal antibodies.

The number of total T-cells and the number of helper/inducer T-cells decreased in 27 of 31 patients with immunologic disorders treated with plasmapheresis and drug immunosuppression. The number of suppressor/cytotoxic T-cells increased in the majority of patients. The helper/inducer to suppressor/cytotoxic cell ratio, which was initially elevated in 26 of 31 patients, decreased significantly in all but 2 patients after therapy. Ten patients were already on drug immunosuppression at the time plasmapheresis was started, and all 10 showed increased helper/inducer to suppressor/cytotoxic cell ratio despite their drug therapy. The helper/inducer to suppressor/cytotoxic cell ratio decreased in all ten patients after plasma exchange. The number and the percentage of total T-cells and helper/inducer cells decreased from prepheresis levels while the number and the percentage of suppressor/cytotoxic cells increased. Plasmapheresis, in combination with drug immunosuppression, was effective in decreasing the elevated helper/inducer to suppressor/cytotoxic cell ratio in disorders of the immune system. In contrast, patients on immunosuppressive drug therapy alone had persistently high immunoregulatory ratio.

Antibodies, Monoclonal↗

PubMed1985

The clinical effectiveness and safety of chronic plasmapheresis in patients with primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative, destructive cholangitis, whose etiology is unknown. Morbidity arises early from pruritus and later from hypercholesterolemia with xanthoma formation. Therapy is supportive and directed at the complications of cholestasis. Plasmapheresis has been reported to benefit patients with hyperlipidemia and PBC; thus a pilot study of plasmapheresis utilizing the Haemonetics Model 30 with replacement by albumin and saline was conducted. Five patients (four female and one male) with a mean age of 43 (range 29-58) and a mean duration of illness of 9.5 years (range 6-21) with marked jaundice, xanthomas, xanthelasma, hepatomegaly, fatigability, anorexia, and pruritus, as well as mild nausea were studied. Peripheral neuropathy was present in two patients. Two patients had splenomegaly. Two patients had an associated Sjogren syndrome. All patients had high serum bilirubin, alkaline phosphatase, and cholesterol levels and mild elevations in aspartate amino transferase and alanine amino transferase activities. Immune complexes measured in four patients were present. Antimitochondrial antibody titers were significant in all patients. Patients underwent a mean of 63 plasmapheresis procedures over a mean of 112 weeks removing a mean of 94.7 liters of plasma. No serious toxicity was seen. All patients showed a reduction in pruritus, xanthomas, xanthelasmas, and serum cholesterol values. The two patients who had evidence of Sjogren syndrome noted subjective improvement. All patients who had fatigue, anorexia and nausea also noted moderate improvement. There was no change in hepatomegaly or splenomegaly in patients demonstrating such organomegaly. Liver function did not change significantly. Overall, four patients had improvement in their condition and one patient achieved stability.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1987

Clinical evaluation of three types of plasmapheresis in a patient with type IIa familial hypercholesterolemia.

Long-term plasmapheresis (PP) therapy was studied in a 56-year-old patient with homozygous type IIa familial hypercholesterolemia also suffering from severe coronary heart disease. Three different PP techniques, plasma exchange (PE), double-membrane-filtration plasmapheresis (DFP), and the recently developed low-density lipoprotein adsorbent column (adsorption plasmapheresis, adsorption PP), were used in an attempt to develop better means of managing the disease. All three techniques showed similar elimination efficiency with respect to plasma total cholesterol level. Adsorption PP with minimal supplemental plasma protein managed the circulatory status of the patient better than DFP during extracorporeal treatment. In the course of PP therapy xanthoma tuberosum markedly regressed, and the cardiac status of the patient was clearly improved.

Angiography↗