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김수현,김우준,허소영,이규임,정인자,김호진 대한신경과학회 2013 Journal of Clinical Neurology Vol.9 No.1
Background and Purpose Although plasmapheresis is becoming standard practice as a rescue therapy for neuromyelitis optica (NMO), evidence for the therapeutic efficacy of plasmapheresis is limited, and the effect of plasmapheresis on anti-aquaporin-4 (AQP4) levels in patients with NMO has not been reported. Here, our objective was to evaluate the clinical efficacy of therapeutic plasmapheresis and its effect on anti-AQP4 antibody levels in patients with NMO spectrum disorder (NMOSD). Methods We retrospectively reviewed the medical records of 15 patients with NMOSD who had 18 acute attacks and received plasmapheresis because they did not respond to high-dose intravenous methylprednisolone (IVMP) therapy. Anti-AQP4 antibodies were measured before and after plasmapheresis. The primary outcomes were functional improvements immediately and 6 months after plasmapheresis, and the secondary outcome was the change in anti-AQP4antibody serum levels following plasmapheresis. Results Plasmapheresis following IVMP therapy led to significant improvement in 50% of the 18 attacks in 15 patients immediately after the procedure was completed, and in 78% (14attacks) after 6 months. Plasmapheresis was generally well tolerated in all patients. Anti-AQP4antibody serum levels declined significantly following plasmapheresis, to a mean of 15% of the preplasmapheresis levels. Lower scores on the visual outcome scale recorded before an attack were associated with significant immediate improvement upon the completion of plasmapheresis (p=0.03). Conclusions Plasmapheresis following IVMP therapy effectively removed anti-AQP4 antibodies and was accompanied by a substantial improvement in the neurological disability of patients with NMOSD. Lower levels of pre-existing neurological damage may be associated with an improved acute response to plasmapheresis.
신채원,김수현,조소영,이광우,장준영,김성민 대한신경과학회 2009 대한신경과학회지 Vol.27 No.4
Plasmapheresis is an emerging treatment for intravenous steroid-resistant neuromyelitis optica (NMO). We present the case of a 16-year-old girl who suffered from intravenous steroid-resistant NMO and whose neurological status improved markedly after treatment with plasmapheresis. This is the first report on the effectiveness of plasmapheresis in NMO in Korea. Plasmapheresis is an emerging treatment for intravenous steroid-resistant neuromyelitis optica (NMO). We present the case of a 16-year-old girl who suffered from intravenous steroid-resistant NMO and whose neurological status improved markedly after treatment with plasmapheresis. This is the first report on the effectiveness of plasmapheresis in NMO in Korea.
Alireza Sadeghi,Somayeh Sadeghi,Mohammad Saleh Peikar,Maryam Yazdi,Mehran Sharifi,Safie Ghafel,Farzin Khorvash,Behrooz Ataei,Mohammad Reza Safavi,Elahe Nasri 대한혈액학회 2023 Blood Research Vol.58 No.2
Background With the emergence of the coronavirus disease 2019 (COVID-19) and inability of healthcare systems to control the disease, various therapeutic theories with controversial responses have been proposed. Plasmapheresis was administered as a medication. However, the knowledge of its efficacy and indications is inadequate. This study evaluated the use of plasmapheresis in critically ill patients with cancer. Methods This randomized clinical trial was conducted on 86 patients with malignancies, including a control group (N=41) and an intervention group (N=45) with severe COVID-19 during 2020-21. Both groups were treated with routine medications for COVID-19 management according to national guidelines, and plasmapheresis was applied to the intervention group. C-reactive protein (CRP), D-dimer, ferritin, lactate dehydrogenase, hemoglobin, and white blood cell, polymorphonuclear, lymphocyte, and platelet levels were measured at admission and at the end of plasmapheresis. Other variables included neutrophil recovery, intensive care unit admission, intubation requirements, length of hospital stay, and hospitalization outcomes. Results CRP (P <0.001), D-dimer (P <0.001), ferritin (P =0.039), and hemoglobin (P =0.006) levels were significantly different between the groups after the intervention. Neutrophil recovery was remarkably higher in the case than in the control group (P <0.001). However, plasmapheresis did not affect the length of hospital stay (P=0.076), which could have significantly increased survival rates (P <0.001). Conclusion Based on the study findings, plasmapheresis led to a significant improvement in laboratory markers and survival rate in patients with severe COVID-19. These findings reinforce the value of plasmapheresis in cancer patients as a critical population suffering from neutropenia and insufficient immune responses.
Boram Lee,Jai Young Cho,Hae Won Lee,최영록,윤유석,한호성 대한이식학회 2020 Korean Journal of Transplantation Vol.34 No.2
The role of the isoagglutinin (IA) titer in liver transplantation (LT) is still not well defined, but the general belief is that a higher titer may result in a higher risk of rejection in ABO-incompatible living donor LT. To reduce the IA titer by 1:16 or lower, plasmapheresis is usually performed before transplantation. However, there is no established protocol for patients for whom plasmapheresis has failed before reaching the target IA titers. Here, we report the cases of three patients who show high baseline IA titers and have failed plasmapheresis: no-response to plasmapheresis, allergic reaction associated with plasmapheresis, and anaphylactic reaction to platelet transfusion. For various reasons, after several plasmapheresis procedures, IA titers were not effectively reduced. In these patients, splenectomy and intravenous immunoglobulin (0.8 g/kg, from the anhepatic phase to 2 days after transplantation) were carried. The protocol biopsy on postoperative day 7 showed no histologic evidence of meaningful acute rejection. The main aim of this work is to demonstrate that we can apply this protocol to patients who have high baseline IA titers and have failed plasmapheresis. Furthermore, this report is enhanced to promote to the transplant community this approach with this type of recipient.
A 1-month-old infant with chylomicronemia due to GPIHBP1 gene mutation treated by plasmapheresis
정모경,진주현,김현옥,권아름,채현욱,강석진,김덕희,김호성 대한소아내분비학회 2017 Annals of Pediatirc Endocrinology & Metabolism Vol.22 No.1
Chylomicronemia is a severe type of hypertriglyceridemia characterized by chylomicron accumulation that arises from a genetic defect in intravascular lipolysis. It requires urgent and proper management, because serious cases can be accompanied by pancreatic necrosis or persistent multiple organ failure. We present the case of a 1-month-old infant with chylomicronemia treated by plasmapheresis. His chylomicronemia was discovered incidentally when lactescent plasma was noticed during routine blood sampling during a hospital admission for fever and irritability. Laboratory investigation revealed marked triglyceridemia (>5,000 mg/dL) with high chylomicron levels. We therefore decided to perform a therapeutic plasmapheresis to prevent acute pancreatitis. Sequence analysis revealed a homozygous novel mutation in exon 4 of GPIHBP1: c.476delG (p.Gly159Alafs). Glycosylphosphatidylinositol-anchored high density lipoproteinbinding protein 1 (GPIHBP1) stabilizes the binding of chylomicrons near lipoprotein lipase and supports lipolysis. Mutations of GPIHBP1, the most recently discovered gene, can lead to severe hyperlipidemia and are known to make up only 2% of the monogenic mutations associated with chylomicronemia. The patient maintains mild hypertriglyceridemia without rebound after single plasmapheresis and maintenance fibrate medication so far. Here, we report an infant with chylomicronemia due to GPIHBP1 mutation, successfully treated by plasmapheresis.
Cho, Jang-Hee,Lee, Jong-Hak,Park, Ga-Young,Lim, Jeong-Hoon,Kim, Jun-Seop,Kang, Yoon-Jung,Kwon, Owen,Choi, Ji-Young,Park, Sun-Hee,Kim, Yong-Lim,Kim, Hyung-Kee,Huh, Seung,Kim, Chan-Duck Informa Healthcare USA, Inc. 2014 Renal failure Vol.36 No.4
<P>Recurrence of focal segmental glomerulosclerosis (FSGS) is a major therapeutic challenge in kidney transplantation (KT). Although intensive plasmapheresis and high-dose rituximab have been introduced to treat recurrent FSGS, the most effective dosage and regimen of rituximab have not been determined. Herein we reported the first case of successful treatment of recurrent FSGS with a low-dose rituximab. The patient showed marked proteinuria (3.5 g/d) and oliguria 2 d after KT. Two courses of plasmapheresis and immunoglobulin were applied to the patient, however, nephrotic range proteinuria persisted and creatinine level increased to 3.56 mg/dL. Five months post-transplant, the patient received injection with only one dose of rituximab 100 mg, without further plasmapheresis, which resulted in immediate reduction of serum creatinine and full remission of proteinuria during the following 18 months. This case suggested that recurrent FSGS, which frequently relapses after plasmapheresis, could be treated successfully with a low-dose rituximab even without plasmapheresis.</P>
장기간 집중적인 혈장반출법으로 치유된 mitomycin C에 의한 용혈요독증후군
신동호 ( Dong Ho Shin ),이미정 ( Mi Jung Lee ),박현성 ( Hyun Sung Park ),김좌경 ( Jwa Kyung Kim ),박정탁 ( Jung Tak Park ),장태익 ( Tae Ik Chang ),강신욱 ( Shin Wook Kang ),최규헌 ( Kyu Hun Choi ),유태현 ( Tae Hyun Yoo ) 대한신장학회 2010 Kidney Research and Clinical Practice Vol.29 No.5
HUS (Hemolytic Uremic Syndrome) is characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. In classical HUS, hemorrhagic diarrhea precedes. It is frequently associated with E. Coli O157:H7. Less frequently, HUS may also develop after various treatments such as mitomycin C, cyclosporine, quinine, and ticlopidine. Plasmapheresis is effective in most of classical HUS, which induces a complete remission in most patients with classical HUS. However, this treatment is ineffective in HUS associated with mitomycin C. Although Plasmapheresis is effective on hematologic abnormality in this atypical HUS, chronic renal insufficiency frequently persists as a sequella in HUS associated with mitomycin C. We here report on one patient who developed HUS following mitomycin C therapy due to cervix cancer. The patient was treated with intensive and prolonged plasmapheresis. There was a complete hematologic improvement and steady improvement in renal function.
미만성 폐포출혈을 동반한 전신성 홍반성 루푸스 환자에서 Plasmapheresis를 이용한 치험
고재현 ( Jay Hyun Koh ),송서영 ( Seo Young Song ),이창근 ( Chang Keun Lee ),서기현 ( Gi Hyeon Seo ),안홍준 ( Hong Joon Ahn ),차훈석 ( Hoon Suk Cha ),김진석 ( Jin Seok Kim ),고은미 ( Eun Mi Koh ),송재훈 ( Jae Hoon Song ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.2
Pulmonary alveolar hemorrhage (PAH) is a rare and often fatal presenting feature of systemic lupus erythematosus (SLE) and enters the differential diagnosis of diffuse lung disease in patients with SLE. Reported mortality rates are extremely high, between 70 and 90 percents. Because death frequently occurs within the first several days of the hemorrhage, the diagnosis needs to be established promptly and treatment should be initiated immediately. Treatment of alveolar hemorrhage has included various combinations of corticosteroids, cytotoxic agents, and plasmapheresis, but survival rates have been extremely low despite aggressive therapy. We experienced a case of diffuse alveolar hemorrhage in a 29 year-old SLE male patient. PAH was diagnosed by hemoptysis, anemia, infiltration on chest X-ray and hemosiderin-laden macrophages in bronchoalveolar lavage. After high dose intravenous steroid, cyclophosphamide intravenous therapy and plasmapheresis, the condition of patient was markedly improved. He was discharged and received monthly intravenous pulse cyclophosphamide. He has done well since, showing no further pulmonary hemorrhage with steroid tapering.