Journal of Clinical Apheresis最新文献

{"title":"Botulism mimicking Guillain-Barre syndrome: The question of plasma exchange in an unusual case of acute paralysis","authors":"Juan J. Silva Campos MD,&nbsp;Elizabeth Abels MD,&nbsp;Henry M. Rinder MD,&nbsp;Christopher A. Tormey MD,&nbsp;Jeremy W. Jacobs MD, MHS","doi":"10.1002/jca.22081","DOIUrl":"10.1002/jca.22081","url":null,"abstract":"<p>Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy and the most common cause of acute flaccid paralysis worldwide. GBS classically presents with acute, progressive, ascending weakness, reduced to absent reflexes, and albuminocytological dissociation on cerebrospinal fluid (CSF) analysis. Botulism is a neurotoxin-mediated acute descending flaccid paralysis with cranial nerve palsies and dysautonomia. Botulism in adults is caused by ingestion/inhalation of botulinum toxin or wound infection with <i>Clostridium botulinum</i>. Both GBS and botulism can rapidly precipitate respiratory failure; thus, prompt diagnosis and treatment are crucial to mitigate poor outcomes. Herein, we describe a case of botulism initially diagnosed as GBS given classic laboratory features, and describe the importance of careful consideration of the most appropriate therapeutic modalities in cases of acute flaccid paralysis, particularly regarding empiric administration of botulinum antitoxin and use of intravenous immune globulin in lieu of plasma exchange for potential GBS to prevent removal of antitoxin.</p>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 6","pages":"760-763"},"PeriodicalIF":1.5,"publicationDate":"2023-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9897852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Stem cell mobilizating effect of heparin in patients undergoing autologous stem cell transplantation","authors":"Mustafa Merter MD,&nbsp;Ugur Sahin MD,&nbsp;Osman İlhan MD,&nbsp;Meral Beksac MD","doi":"10.1002/jca.22079","DOIUrl":"10.1002/jca.22079","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Adequate stem cell collection is essential for successful stem cell transplantation. Heparin enhances stem cell mobilization by competing with heparin sulfate proteoglycans. Heparin is also used as an anticoagulant before leukapheresis. Here, we evaluated the effects of heparin on stem cell mobilization in patients who underwent autologous stem cell transplantation (ASCT).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We evaluated patients who underwent ASCT. Patients were divided into two groups: those who received heparin plus citrate (heparinized patients) and those who received citrate only (nonheparinized patients) for anticoagulation. Univariate and multivariate analyses were also performed. The collection efficiency 2 (CE2) for CD34+ cells was calculated and compared between heparinized and nonheparinized patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>This study included 1017 patients. There were 478 (47%) heparinized and 539 (53%) nonheparinized patients. The number of collected CD34+ cells was significantly higher in heparinized patients (<i>P</i> &lt; .00001). The multivariate analyses showed that using heparin was an independent positive factor for collected CD34+ cells (adj-<i>R</i>² = 0.744; <i>F</i> = 369.331, <i>P</i> &lt; .00001). CE2 was significantly higher in heparinized patients than in nonheparinized patients (66.8% vs 52.1%; <i>P</i> &lt; .00001). The rate of collecting at least 2 × 10⁶/kg CD34+ cells was 3.3 times higher for heparinized patients in poor mobilizers (<i>P</i> &lt; .00001). Heparinized patients had significantly higher total nucleated and mononuclear cell counts (<i>P</i> &lt; .00001 and &lt;.00001, respectively).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Heparin enhances stem cell collection and increases CE2. The use of heparin may reduce the need for other strategies to increase stem cell mobilization.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 6","pages":"685-693"},"PeriodicalIF":1.5,"publicationDate":"2023-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9886664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development and validation of a nomogram for predicting the risk of vasovagal reactions after plasma donation","authors":"Peizhe Zhao MS,&nbsp;Demei Dong MD,&nbsp;Rong Dong MD,&nbsp;Yuan Zhou MD,&nbsp;Yan Hong MD,&nbsp;Guanglin Xiao MS,&nbsp;Zhiye Li MS,&nbsp;Xuelin Su BS,&nbsp;Xingyou Zheng BS,&nbsp;Xia Liu BS,&nbsp;Demei Zhang MD,&nbsp;Ling Li MD,&nbsp;Zhong Liu MD","doi":"10.1002/jca.22074","DOIUrl":"10.1002/jca.22074","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Objectives</h3>\u0000 \u0000 <p>Vasovagal reactions (VVRs) are the most common adverse reactions and are frequently associated with serious donor adverse events. Even mild VVRs can lead to a significant reduction in the likelihood of subsequent donations. The purpose of this study is to explore the factors related to the occurrence of VVRs after plasma donation and to construct a nomogram to identify individuals at risk for VVRs to improve the safety of plasma donors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>We collected the donation data from July 2019 to June 2020 from a plasma center in Sichuan, China, to explore the independent risk factors for vasovagal reactions. From these data, we constructed and validated a predictive model for vasovagal reactions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>VVRs after plasma donation occurred 737 times in 120 448 plasma donations (0.66%). Gender, season, donor status, weight, pulse, duration of donation, and cycle were independent risk factors for VVRs (<i>P</i>&lt; 0.05). The concordance index (C-index) of a logistic model in the derivation cohort was 0.916, with a Hosmer-Lemeshow goodness-of-fit probability of 0.795. The C-index of a logistic model in the validation cohort was 0.916, with a Hosmer-Lemeshow goodness-of-fit probability of 0.224. The calibration curve showed that the predicted results were in good agreement with the actual observed results.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This study preliminarily constructed and verified a prediction model for VVRs after plasma donation. The model nomogram is practical and can identify high-risk donors.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"622-631"},"PeriodicalIF":1.5,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9886846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Extracorporeal photopheresis induces NETosis in neutrophils derived from patients with chronic graft-vs-host disease","authors":"Idan Goldberg,&nbsp;Galit Granot,&nbsp;Alona Telerman,&nbsp;Shirly Partouche,&nbsp;Tzippy Shochat,&nbsp;Erez Halperin,&nbsp;Anat Gafter-Gvili,&nbsp;Liat Shargian,&nbsp;Moshe Yeshurun,&nbsp;Pia Raanani,&nbsp;Ofir Wolach,&nbsp;Vered Yahalom","doi":"10.1002/jca.22073","DOIUrl":"10.1002/jca.22073","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Extracorporeal photopheresis (ECP) is considered an effective treatment for patients with chronic graft vs host disease (cGVHD) and demonstrates efficacy in ameliorating GVHD. The mechanism by which ECP acts against cGVHD is not fully understood. Preliminary observations have hinted at the potential involvement of neutrophil extracellular traps (NETs) formation in the pathogenesis of cGVHD. We aimed to assess the influence of ECP on the formation of NETs in patients with cGVHD as a potential mechanism in this setting.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients treated with ECP for cGVHD at the Rabin Medical Center were included in this study. Blood samples were obtained at three different time points: before starting an ECP cycle, at the end of the first day of treatment, and 24 h following the initiation of the ECP treatment cycle. Neutrophils were harvested from all blood samples. NET formation was assessed by measurement of NET-bound specific neutrophil elastase activity and by immunofluorescence staining.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Six patients (two females and four males) with cGVHD were included in the study. We observed a significant increase in NET formation among all six patients following ECP. Net-bound specific neutrophil elastase activity was elevated from a median value of 2.23 mU/mL (interquartile range [IQR] 2.06-2.47 mU/mL) at baseline to a median value of 13.06 mU/mL (IQR 10.27-15.97 mU/mL) immediately after the treatment and to a peak median value of 14.73 mU/mL (IQR 9.6-22.38 mU/mL) 24 h following the initiation of the ECP cycle. A qualitative assessment of NET formation using immunofluorescence staining has demonstrated markedly increased expression of citrullinated histone H3, a marker of NET formation, following ECP treatment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our preliminary data indicate that ECP induces NET formation among patients with cGVHD. The contribution of increased NET formation to the therapeutic effect of cGVHD should be further investigated.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"615-621"},"PeriodicalIF":1.5,"publicationDate":"2023-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jca.22073","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9829638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of extracorporeal photopheresis on survival in acute graft versus host disease","authors":"Ahmet Kaya,&nbsp;Mehmet Ali Erkurt,&nbsp;İrfan Kuku,&nbsp;Emin Kaya,&nbsp;İlhami Berber,&nbsp;Soykan Biçim,&nbsp;Emine Hidayet,&nbsp;Salih Cırık,&nbsp;Süleyman Arslan,&nbsp;Fatma Hilal Yagın,&nbsp;Ahmet Sarıcı","doi":"10.1002/jca.22071","DOIUrl":"10.1002/jca.22071","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Extracorporeal photopheresis (ECP) is the main non-pharmacological approach accompanying systemic medical treatments in steroid-resistant acute or chronic graft versus host disease. The study aimed to examine the effect of ECP on survival in acute graft versus host disease (aGVHD).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A total of 35 patients who were followed up in the adult hematology clinic of İnönü University Turgut Özal Medical Center for aGVHD were included in the study. Stem cell transplantation and ECP application parameters that may affect the survival of the patients were examined.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In aGVHD using ECP, the degree of involvement affects survival. Involvements with a clinical and laboratory score (Glucksberg system) of 2 and above significantly reduced survival. The duration of ECP use is associated with survival. Especially, 45 days and longer use increases survival (hazard ratio, <i>P</i>-value &lt;.05). The duration of steroid use was found to be effective in survival in aGVHD (<i>P</i> &lt; .001). ECP administration day (<i>P</i> = .003), duration of steroid use (<i>P</i> &lt; .001), duration of ECP use (<i>P</i> = .001), and grade of aGVHD (<i>P</i> &lt; .001) affect survival.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>ECP use is effective in survival in patients with aGVHD score ≥2. In patients with aGVHD, especially the use of 45 days and longer has a positive effect on survival. The duration of steroid use is associated with survival in aGVHD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"602-610"},"PeriodicalIF":1.5,"publicationDate":"2023-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9758832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficient removal of antibodies to adeno-associated viruses by immunoadsorption","authors":"Simone Boedecker-Lips,&nbsp;Andreas Judel,&nbsp;Stefan Holtz,&nbsp;Magnus Mayer,&nbsp;Pascal Klimpke,&nbsp;Daniel Kraus,&nbsp;Thomas Schreiner,&nbsp;Bernhard Gerstmayer,&nbsp;Klaus Eulitz,&nbsp;Magnus Christopher Mayer,&nbsp;Julia Weinmann-Menke","doi":"10.1002/jca.22069","DOIUrl":"10.1002/jca.22069","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Gene therapies based on adeno-associated viruses (AAV) are a therapeutic option to successfully treat monogenetic diseases. However, the influence of pre-existing immunity to AAV can compromise the application of AAV gene therapy, most notably by the presence of neutralizing antibodies (NAb) to AAV.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In the following study, we investigated to what extent the treatment by immunoadsorption (IA) would reduce the levels of human anti-AAV antibodies to AAV2 and AAV5. To that end, we screened blood sera from 40 patients receiving IA treatment because of underlying autoimmune disease or transplant rejection, with detectable AAV-antibodies in 23 patients (22 by NAb detection, and 1 additionally by anti-AAV5 ELISA analysis).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Our results show that IA efficiently depleted anti-AAV2 NAb with a mean reduction of 3.92 ± 1.09 log2 titer steps (93.4%) after three to five single IA treatments, 45% of seropositive subjects had an anti-AAV2 titer below the threshold titer of 1:5 after the IA treatment series. Anti-AAV5 NAb were reduced to below the threshold titer of 1:5 in all but one of five seropositive subjects. Analysis of total anti-AAV5 antibodies by ELISA demonstrated an anti-AAV5 antibody reduction over the IA treatment series of 2.67 ± 1.16 log2 titer steps (84.3%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In summary, IA may represent a safe strategy to precondition patients with pre-existing anti-AAV antibodies to make this population eligible for an effective AAV-based gene therapy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"590-601"},"PeriodicalIF":1.5,"publicationDate":"2023-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jca.22069","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9761726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Therapy plans for therapeutic apheresis in Epic HealthLink","authors":"Vansh S. Jain,&nbsp;Huihua Li,&nbsp;Kristin P. Lee,&nbsp;William Nicholas Rose","doi":"10.1002/jca.22072","DOIUrl":"10.1002/jca.22072","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>A common required duty of pathology resident physicians while rotating on transfusion medicine is the medical oversight of the therapeutic apheresis service. A task often performed on this clinical medicine service is formulating and writing orders for therapeutic apheresis procedures. The EpicCare tool called the therapy plan provides unique advantages over a standard electronic order set for therapeutic apheresis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>Transfusion medicine physicians, apheresis nurses, pharmacists, and information technology professionals collaborated to create therapy plans for three therapeutic apheresis procedures: plasmapheresis, red cell exchange, and photopheresis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Therapy plans were implemented and have been well-received for several years. Over a six-year time period, a total of 613 therapy plans were created and signed. We speculate that this implementation may have increased both physician efficiency and patient safety.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This article reports our experience using therapy plans in EpicCare in order to raise awareness of this tool and to serve as an encouragement for wider adoption.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"611-614"},"PeriodicalIF":1.5,"publicationDate":"2023-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jca.22072","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9695932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Why has plasma exchange failed in TRACK syndrome? Lessons from a new variant of the atypical hemolytic uremic syndrome","authors":"Cansu Durak,&nbsp;Ebru Guney Sahin,&nbsp;Yasar Yusuf Can,&nbsp;Fatih Varol,&nbsp;Halit Cam","doi":"10.1002/jca.22070","DOIUrl":"10.1002/jca.22070","url":null,"abstract":"<p>Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening form of thrombotic microangiopathy, associated with high mortality and morbidity. Most cases present with hemolytic anemia, thrombocytopenia, and renal insufficiency. However, it can have unusual multiple end-organ injuries including extrarenal organ and system involvements such as neurologic, cardiac, gastrointestinal, and respiratory systems. We describe a 4-year-old girl who developed aHUS due to the TSEN2 mutation and had cardiac involvement. She did not benefit from plasma exchange, as stated in previous cases. It should be kept in mind that therapeutic plasma exchange may not be beneficial in some cases of aHUS, especially due to genetic mutations.</p>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"647-650"},"PeriodicalIF":1.5,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9667837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A validation and modification of PLASMIC score by adjusting the criteria of mean corpuscular volume and international normalized ratio","authors":"Jia-Arng Lee MD, MSc,&nbsp;Mei-Hwa Lin BS,&nbsp;Chun-Min Kang MD,&nbsp;Ming-Kai Chuang MD,&nbsp;Chi Kwan Boris Fung MD,&nbsp;Shyh-Chyi Lo MD, PhD","doi":"10.1002/jca.22068","DOIUrl":"10.1002/jca.22068","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The PLASMIC score was developed for distinguishing thrombotic thrombocytopenic purpura (TTP) from other types of thrombotic microangiopathy. However, two components of the PLASMIC score, mean corpuscular volume (MCV) and international normalized ratio (INR), showed non-significant differences between TTP and non-TTP patients in previous validations. Here, we validate the PLASMIC score and aim to modify it by adjusting the criteria of MCV and INR.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>A retrospective validation of suspected TTP patients was performed by reviewing electronic medical records from two medical centers in Taiwan. The performance of different modified types of the PLASMIC score was carried out.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 50 patients included in the final analysis, 12 were diagnosed with TTP based on deficiency of ADAMTS13 activity and clinical judgement. When stratified by high (score ≥ 6) and low-intermediate risk (score &lt; 6), the positive predictive value (PPV) of the PLASMIC score to predict TTP was 0.45 (95% confidence interval [CI]: 0.29-0.61). The area under curve (AUC) was 0.70 (95% CI: 0.56-0.82). When adjusting the criteria of the PLASMIC score from MCV &lt; 90 fL to MCV ≥ 90 fL, the PPV increased to 0.57 (95% CI: 0.37-0.75). The AUC was 0.75 (95% CI: 0.61-0.87). When adjusting the INR from &gt;1.5 to &gt;1.1, the PPV increased to 0.56 (95% CI: 0.39-0.71). The AUC was 0.81 (95% CI: 0.68-0.90).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>MCV ≥ 90 fL and/or INR &gt; 1.1 might be suitable modifications for PLASMIC score but should be validated in a larger sample size.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"582-589"},"PeriodicalIF":1.5,"publicationDate":"2023-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9995571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry","authors":"Osman Radhwi MD,&nbsp;Maha A. Badawi MD,&nbsp;Adel Almarzouki MD,&nbsp;Fakhr Al-Ayoubi PhD,&nbsp;Ghada ElGohary MD,&nbsp;Kazi Nur Asfina MD,&nbsp;Abdulrahim Mohammed Basendwah MD,&nbsp;Iman Ayed Alhazmi MD,&nbsp;Eiman A. Almahasnah MD,&nbsp;Ahmed AlBahrani MD,&nbsp;Abdulrahman Al Raizah MD,&nbsp;Ayel Yahya MD,&nbsp;Khadeja Alshahrani MD,&nbsp;Salwa Hindawi MD","doi":"10.1002/jca.22067","DOIUrl":"10.1002/jca.22067","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>One hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 10⁹/L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"38 5","pages":"573-581"},"PeriodicalIF":1.5,"publicationDate":"2023-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9632347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}