Multiple myeloma associated with bleeding events secondary to von Willebrand syndrome is underdiagnosed. The management of this entity is highly complex, and aims to control the hemorrhagic syndrome and reduce plasma viscosity with plasmapheresis and multiple myeloma-specific treatment. The authors report the rare case of a young patient with high-risk multiple myeloma complicated by hyperviscosity syndrome and presenting an acquired von Willebrand syndrome with hemorrhagic manifestations, requiring urgent therapeutic management to save the patient's life.
{"title":"Management of a Young Patient With High-risk Multiple Myeloma Complicated by Acquired von Willebrand Syndrome: A Diagnostic and Therapeutic Emergency","authors":"Abakarim Ouadii, Condom Pauline, Voisin Sophie, Huart Antoine, Perrot Aurore, Botin Teresa","doi":"10.1177/26348535231222994","DOIUrl":"https://doi.org/10.1177/26348535231222994","url":null,"abstract":"Multiple myeloma associated with bleeding events secondary to von Willebrand syndrome is underdiagnosed. The management of this entity is highly complex, and aims to control the hemorrhagic syndrome and reduce plasma viscosity with plasmapheresis and multiple myeloma-specific treatment. The authors report the rare case of a young patient with high-risk multiple myeloma complicated by hyperviscosity syndrome and presenting an acquired von Willebrand syndrome with hemorrhagic manifestations, requiring urgent therapeutic management to save the patient's life.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"114 6","pages":""},"PeriodicalIF":0.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139393930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-01-01DOI: 10.1177/26348535231225315
Rohollah Baktashi, Fariba Rad, Masoud Torabi Ardekani, Alireza Salah, Hossein Anani, R. M. Khalilabadi
In Iran, blood products were being delivered to healthcare centers without charge. For optimum usage and reducing wastage, the tariff was set on October 23, 2015. Thus, hospitals must pay for blood products to blood transfusion services and then the expenses are paid to healthcare centers by the insurance. This study aimed to explore the effects of the tariff policy on utilization pattern of blood products in healthcare centers of Fars province, Iran. This is a retrospective study which analyzes the recorded data. Throughout this retrospective study, the number of units requested, delivered, returned, and return/delivery ratio of RBC, FFP, cryoprecipitate, and platelet were examined before and after the tariff in different cities of Fars province of Iran. After the tariff, return/delivery ratios of FFP, cryoprecipitate, and platelet increased in comparison with before tariff but were not statistically significant which means the return of unused units was increased after imposing tariff. The results of this investigation showed that tariff may lead to more return of FFP, cryoprecipitate, and platelet units to the transfusion services of Fars province. More research must be performed to further approve it.
{"title":"Effects of Tariff on the Management of Blood Products in Fars Province Hospitals","authors":"Rohollah Baktashi, Fariba Rad, Masoud Torabi Ardekani, Alireza Salah, Hossein Anani, R. M. Khalilabadi","doi":"10.1177/26348535231225315","DOIUrl":"https://doi.org/10.1177/26348535231225315","url":null,"abstract":"In Iran, blood products were being delivered to healthcare centers without charge. For optimum usage and reducing wastage, the tariff was set on October 23, 2015. Thus, hospitals must pay for blood products to blood transfusion services and then the expenses are paid to healthcare centers by the insurance. This study aimed to explore the effects of the tariff policy on utilization pattern of blood products in healthcare centers of Fars province, Iran. This is a retrospective study which analyzes the recorded data. Throughout this retrospective study, the number of units requested, delivered, returned, and return/delivery ratio of RBC, FFP, cryoprecipitate, and platelet were examined before and after the tariff in different cities of Fars province of Iran. After the tariff, return/delivery ratios of FFP, cryoprecipitate, and platelet increased in comparison with before tariff but were not statistically significant which means the return of unused units was increased after imposing tariff. The results of this investigation showed that tariff may lead to more return of FFP, cryoprecipitate, and platelet units to the transfusion services of Fars province. More research must be performed to further approve it.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"101 9","pages":""},"PeriodicalIF":0.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139454120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231171332
Ilaria Ramaiola, Laura Lopez, Manuel Moron, Pau Maduell, Maite López, Nuria Marzo, Salvador Grancha
Background The activity and purity of Fibrin Sealant Grifols are described in this article. This fibrin sealant contains two components (fibrinogen and thrombin) that mimic the final stages of clotting. Fibrin sealants are primarily used as an adjunct to hemostasis for mild to moderate surgical bleeding in adults when standard surgical techniques are ineffective or impractical. Objective The aim of these studies was to assess the biochemical and functional characteristics of Fibrin Sealant Grifols. Methods Total protein, plasma protein impurities, and molecular forms were analyzed for both components. Clottable fibrinogen and thrombin activity were also measured. Both components were analyzed by SDS-PAGE and Western blot. The fibrin sealant clot was functionally characterized. Results The components had minimal contamination by other plasma proteins and no aggregates or degradation products. The fibrinogen component was almost completely clottable fibrinogen. The thrombin component contains albumin. The fibrinogen component formed a fibrin polymer and clots with tensile strength similar to literature values. The application device applied the components in a 1:1 volume ratio. Clotting time was ∼ 5 s when applied with the drip tip and nearly instantaneous when applied with the airless spray apparatus. Conclusion This fibrin sealant was shown to contain highly purified fibrinogen and thrombin delivered in a 1:1 volume ratio. These properties contribute to the clinical safety and efficacy of this product for many applications.
{"title":"Characterization of the Components and Functional Properties of a Plasma-Derived Fibrin Sealant","authors":"Ilaria Ramaiola, Laura Lopez, Manuel Moron, Pau Maduell, Maite López, Nuria Marzo, Salvador Grancha","doi":"10.1177/26348535231171332","DOIUrl":"https://doi.org/10.1177/26348535231171332","url":null,"abstract":"Background The activity and purity of Fibrin Sealant Grifols are described in this article. This fibrin sealant contains two components (fibrinogen and thrombin) that mimic the final stages of clotting. Fibrin sealants are primarily used as an adjunct to hemostasis for mild to moderate surgical bleeding in adults when standard surgical techniques are ineffective or impractical. Objective The aim of these studies was to assess the biochemical and functional characteristics of Fibrin Sealant Grifols. Methods Total protein, plasma protein impurities, and molecular forms were analyzed for both components. Clottable fibrinogen and thrombin activity were also measured. Both components were analyzed by SDS-PAGE and Western blot. The fibrin sealant clot was functionally characterized. Results The components had minimal contamination by other plasma proteins and no aggregates or degradation products. The fibrinogen component was almost completely clottable fibrinogen. The thrombin component contains albumin. The fibrinogen component formed a fibrin polymer and clots with tensile strength similar to literature values. The application device applied the components in a 1:1 volume ratio. Clotting time was ∼ 5 s when applied with the drip tip and nearly instantaneous when applied with the airless spray apparatus. Conclusion This fibrin sealant was shown to contain highly purified fibrinogen and thrombin delivered in a 1:1 volume ratio. These properties contribute to the clinical safety and efficacy of this product for many applications.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135649639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231191440
Chanel Morris, Johan Potgieter, Janette Bester
Background Haemophilia A (HA) is a bleeding disorder, due to a deficiency in factor VIII (FVIII). These patients are unable to produce a stable fibrin clot in the propagation phase of coagulation as they do not generate sufficient thrombin. The primary treatment for HA in South Africa remains replacement therapy with standard half-life FVIII clotting factor concentrate, aimed at reducing bleeding episodes. Objectives To evaluate the effect of varying concentrations of FVIII on whole blood (WB) clot architecture and kinetics during clot formation in patients with severe HA. Design A cross-sectional study where blood from 20 patients with HA was exposed to FVIII ex vivo and compared to a control group of 20 healthy individuals. Methods Scanning electron microscopy (SEM) was used to study WB clot architecture and thromboelastography ® (TEG ® ) was used to quantify WB clot kinetics. Results Scanning electron microscopy studies revealed that patients with HA have sparse, disorganized fibrin networks with limited crosslinking and red blood cells (RBCs) stacked in rouleaux formation. Haemophilia A blood spiked to a 10 to 15 IU/dL FVIII concentration showed improvements in the organisation of the fibrin network with some altered RBCs. In addition, blood spiked to a 30 to 35 IU/dL FVIII concentration showed an increase in fibrin formation with normal RBCs. Thromboelastography ® showed that patients with HA had an increased clot initiation time and decreased clot strength. When spiked to a 10 to 15 IU/dL FVIII concentration the clot kinetic profile showed normalization. However, an increase in FVIII concentration higher than 30 IU/dL showed altered clot architecture and kinetics. Conclusion Based on the current study, FVIII levels at 10 to 15 IU/dL improved clot kinetics but did not normalize the architecture. It may be sufficient for prevention of haemorrhages. Factor VIII levels at 30 to 35 IU/dL resulted in rapid but weaker clot formation. However, at this concentration the clot architecture was normalised which is important for haemostasis. Here it was also evident that the findings of these two modalities (TEG ® and SEM) should not be separated but interpreted in conjunction with each other.
{"title":"A Pilot Study Evaluating the <i>Ex Vivo</i> Effects of Varying Factor VIII Concentration on Clot Kinetics and Architecture in Patients With Haemophilia A","authors":"Chanel Morris, Johan Potgieter, Janette Bester","doi":"10.1177/26348535231191440","DOIUrl":"https://doi.org/10.1177/26348535231191440","url":null,"abstract":"Background Haemophilia A (HA) is a bleeding disorder, due to a deficiency in factor VIII (FVIII). These patients are unable to produce a stable fibrin clot in the propagation phase of coagulation as they do not generate sufficient thrombin. The primary treatment for HA in South Africa remains replacement therapy with standard half-life FVIII clotting factor concentrate, aimed at reducing bleeding episodes. Objectives To evaluate the effect of varying concentrations of FVIII on whole blood (WB) clot architecture and kinetics during clot formation in patients with severe HA. Design A cross-sectional study where blood from 20 patients with HA was exposed to FVIII ex vivo and compared to a control group of 20 healthy individuals. Methods Scanning electron microscopy (SEM) was used to study WB clot architecture and thromboelastography ® (TEG ® ) was used to quantify WB clot kinetics. Results Scanning electron microscopy studies revealed that patients with HA have sparse, disorganized fibrin networks with limited crosslinking and red blood cells (RBCs) stacked in rouleaux formation. Haemophilia A blood spiked to a 10 to 15 IU/dL FVIII concentration showed improvements in the organisation of the fibrin network with some altered RBCs. In addition, blood spiked to a 30 to 35 IU/dL FVIII concentration showed an increase in fibrin formation with normal RBCs. Thromboelastography ® showed that patients with HA had an increased clot initiation time and decreased clot strength. When spiked to a 10 to 15 IU/dL FVIII concentration the clot kinetic profile showed normalization. However, an increase in FVIII concentration higher than 30 IU/dL showed altered clot architecture and kinetics. Conclusion Based on the current study, FVIII levels at 10 to 15 IU/dL improved clot kinetics but did not normalize the architecture. It may be sufficient for prevention of haemorrhages. Factor VIII levels at 30 to 35 IU/dL resulted in rapid but weaker clot formation. However, at this concentration the clot architecture was normalised which is important for haemostasis. Here it was also evident that the findings of these two modalities (TEG ® and SEM) should not be separated but interpreted in conjunction with each other.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"30 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135649640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231156848
David Michael Kaylor, Regan Merrell Wade, Kathryn Brinkley Chappell, Meredith Huff Niemann, Vanessa Marie VanArsdale
Background Although reduced doses of enoxaparin have been studied in low body weight patients, no study has directly compared enoxaparin to unfractionated heparin for venous thromboembolism (VTE) prophylaxis in this population. Objective To determine the most safe and efficacious parenteral venous thromboembolism prophylaxis regimen for low body weight patients. Design Retrospective cohort. Methods Hospitalized patients weighing < 55 kg receiving either enoxaparin or unfractionated heparin for VTE prophylaxis over a period of two and a half years were evaluated. Primary outcomes included rates of VTE and overall bleeding events. Secondary outcomes included rates of major and clinically relevant minor bleeding. Dosing regimens were analyzed to determine associations between dose, weight, creatinine clearance, VTE, and bleeding events. Primary and secondary outcomes were evaluated using Chi squared and Fischer's Exact tests. Results Three hundred and sixty-one patients contributed 400 episodes of VTE prophylaxis. Most patients were female (88%), had a Padua score ≥ 4 (82%) and were admitted to medical/surgical units (76%). VTE rates were 0.4% in patients receiving enoxaparin and 2.5% in patients receiving unfractionated heparin ( P = 0.334). Bleeding rates were 12% and 11% in the enoxaparin and unfractionated heparin groups, respectively ( P = 1). Patients receiving enoxaparin 30 mg daily had bleeding rates of 7.6% compared to 14.7% in patients receiving 40 mg daily ( P = 0.134). Patients receiving heparin twice daily had no bleeding events compared to 12.9% of patients receiving heparin three times daily ( P = 0.222). Conclusion Overall, no difference in VTE or bleeding rates were found between patients weighing < 55 kg receiving enoxaparin versus unfractionated heparin, and there was no difference in bleeding or VTE events in the patients receiving enoxaparin 30 mg daily. Due to this, empirically reducing the dose of enoxaparin for VTE prophylaxis appears reasonable and safe in patients weighing < 55 kg.
{"title":"Venous Thromboembolism Prophylaxis with Enoxaparin Versus Unfractionated Heparin in Patients with Low Body Weight","authors":"David Michael Kaylor, Regan Merrell Wade, Kathryn Brinkley Chappell, Meredith Huff Niemann, Vanessa Marie VanArsdale","doi":"10.1177/26348535231156848","DOIUrl":"https://doi.org/10.1177/26348535231156848","url":null,"abstract":"Background Although reduced doses of enoxaparin have been studied in low body weight patients, no study has directly compared enoxaparin to unfractionated heparin for venous thromboembolism (VTE) prophylaxis in this population. Objective To determine the most safe and efficacious parenteral venous thromboembolism prophylaxis regimen for low body weight patients. Design Retrospective cohort. Methods Hospitalized patients weighing < 55 kg receiving either enoxaparin or unfractionated heparin for VTE prophylaxis over a period of two and a half years were evaluated. Primary outcomes included rates of VTE and overall bleeding events. Secondary outcomes included rates of major and clinically relevant minor bleeding. Dosing regimens were analyzed to determine associations between dose, weight, creatinine clearance, VTE, and bleeding events. Primary and secondary outcomes were evaluated using Chi squared and Fischer's Exact tests. Results Three hundred and sixty-one patients contributed 400 episodes of VTE prophylaxis. Most patients were female (88%), had a Padua score ≥ 4 (82%) and were admitted to medical/surgical units (76%). VTE rates were 0.4% in patients receiving enoxaparin and 2.5% in patients receiving unfractionated heparin ( P = 0.334). Bleeding rates were 12% and 11% in the enoxaparin and unfractionated heparin groups, respectively ( P = 1). Patients receiving enoxaparin 30 mg daily had bleeding rates of 7.6% compared to 14.7% in patients receiving 40 mg daily ( P = 0.134). Patients receiving heparin twice daily had no bleeding events compared to 12.9% of patients receiving heparin three times daily ( P = 0.222). Conclusion Overall, no difference in VTE or bleeding rates were found between patients weighing < 55 kg receiving enoxaparin versus unfractionated heparin, and there was no difference in bleeding or VTE events in the patients receiving enoxaparin 30 mg daily. Due to this, empirically reducing the dose of enoxaparin for VTE prophylaxis appears reasonable and safe in patients weighing < 55 kg.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"25 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135649970","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231177667
Maha A. Badawi, Ali Algiraigri, Afnan J. Al-Sulami, Anas A. Abunar, Rami Alharbi, Yoon Soo Park, Ara Tekian
Blood transfusion services maintain quality practice through adherence to guidelines but are faced with blood component wastage due to multiple factors. One factor, in particular, is poor ordering and utilization practices followed by physicians who may not be familiar with the indications and the optimal conditions for the handling of blood products. This study evaluates whether an educational intervention would help reduce ordering of plasma and improve wastage rates after thawing. The study followed a quasi-experimental pre-test post-test design. A multifaceted educational intervention was introduced at a tertiary hospital-based combining predisposing, enabling, and reinforcing activities, based on the literature of effective continuous professional development. The activities included a brief survey to evaluate participant knowledge about indications of plasma and its storage condition, a lecture, and periodic reports and reminders sent to all department heads in the hospital. Respondents to the survey and lecture attendees included physicians in adult critical care and anesthesia departments. Monthly plasma utilization and wastage rates were compared before and after introducing the intervention. Sixty physicians participated in the survey. The questions were answered correctly by less than half. After introducing the intervention, monthly plasma transfusion rates decreased; however, wastage rates did not improve. There is a knowledge gap among physicians who order plasma. The educational intervention resulted in lower plasma transfusion rates but did not affect wastage rates. Future interventions would benefit from including physicians in other departments as well as nurses.
{"title":"A Multifaceted Educational Intervention to Reduce Plasma Utilization","authors":"Maha A. Badawi, Ali Algiraigri, Afnan J. Al-Sulami, Anas A. Abunar, Rami Alharbi, Yoon Soo Park, Ara Tekian","doi":"10.1177/26348535231177667","DOIUrl":"https://doi.org/10.1177/26348535231177667","url":null,"abstract":"Blood transfusion services maintain quality practice through adherence to guidelines but are faced with blood component wastage due to multiple factors. One factor, in particular, is poor ordering and utilization practices followed by physicians who may not be familiar with the indications and the optimal conditions for the handling of blood products. This study evaluates whether an educational intervention would help reduce ordering of plasma and improve wastage rates after thawing. The study followed a quasi-experimental pre-test post-test design. A multifaceted educational intervention was introduced at a tertiary hospital-based combining predisposing, enabling, and reinforcing activities, based on the literature of effective continuous professional development. The activities included a brief survey to evaluate participant knowledge about indications of plasma and its storage condition, a lecture, and periodic reports and reminders sent to all department heads in the hospital. Respondents to the survey and lecture attendees included physicians in adult critical care and anesthesia departments. Monthly plasma utilization and wastage rates were compared before and after introducing the intervention. Sixty physicians participated in the survey. The questions were answered correctly by less than half. After introducing the intervention, monthly plasma transfusion rates decreased; however, wastage rates did not improve. There is a knowledge gap among physicians who order plasma. The educational intervention resulted in lower plasma transfusion rates but did not affect wastage rates. Future interventions would benefit from including physicians in other departments as well as nurses.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135649975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background Immune thrombocytopenia is an immune-mediated disorder with heterogenous presentation. It shows association with other autoimmune disorders with varying frequency. Objective The objective of this study was to establish the frequency of antinuclear antibody (ANA) in our population and to evaluate its association with severity of clinical presentation, disease remission and association with other autoimmune diseases. Design Cross-sectional study. Methods This was a descriptive cross-sectional study that included 160 patients with immune thrombocytopenia purpura (ITP) presented at National Institute of Blood Disease and Bone Marrow Transplantation from February 2021 to July 2022. Patients were screened for ANA and other causes of secondary ITP. ANA-positive patients were compared with ANA-negative patients in terms of bleeding manifestations and platelet counts at initial presentation, association with other autoimmune diseases and response to first-line treatment. Results In our study 43 (26.9%) patients were ANA positive and 117 (73.1%) were negative for ANA. The initial platelet counts, and bleeding symptoms were not found to be significantly different among the 2 groups. And 79.1% of the ANA-positive patients had other autoimmune markers as well compared to 20.9% ANA-negative patients, showing a significant association ( P<.001). Response to treatment with steroid ± azathioprine assessed at 3 and 6 months were similar in both the groups. Conclusion Our results demonstrate that ANA positivity is not associated with severity of presenting symptoms, initial platelet counts, or response to treatment. However, the presence of ANA is associated with increased risk of other autoimmune diseases in patients with ITP.
{"title":"Frequency and Clinical Significance of Antinuclear Antibody Positivity in Immune Thrombocytopenia in Adults","authors":"Rabeea Munawar Ali, Sidra Zafar, Madiha Abid, Aqsa Javed Butt, Muhammad Shujat Ali, Munira Borhany","doi":"10.1177/26348535231165594","DOIUrl":"https://doi.org/10.1177/26348535231165594","url":null,"abstract":"Background Immune thrombocytopenia is an immune-mediated disorder with heterogenous presentation. It shows association with other autoimmune disorders with varying frequency. Objective The objective of this study was to establish the frequency of antinuclear antibody (ANA) in our population and to evaluate its association with severity of clinical presentation, disease remission and association with other autoimmune diseases. Design Cross-sectional study. Methods This was a descriptive cross-sectional study that included 160 patients with immune thrombocytopenia purpura (ITP) presented at National Institute of Blood Disease and Bone Marrow Transplantation from February 2021 to July 2022. Patients were screened for ANA and other causes of secondary ITP. ANA-positive patients were compared with ANA-negative patients in terms of bleeding manifestations and platelet counts at initial presentation, association with other autoimmune diseases and response to first-line treatment. Results In our study 43 (26.9%) patients were ANA positive and 117 (73.1%) were negative for ANA. The initial platelet counts, and bleeding symptoms were not found to be significantly different among the 2 groups. And 79.1% of the ANA-positive patients had other autoimmune markers as well compared to 20.9% ANA-negative patients, showing a significant association ( P<.001). Response to treatment with steroid ± azathioprine assessed at 3 and 6 months were similar in both the groups. Conclusion Our results demonstrate that ANA positivity is not associated with severity of presenting symptoms, initial platelet counts, or response to treatment. However, the presence of ANA is associated with increased risk of other autoimmune diseases in patients with ITP.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"2014 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135649971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231199808
{"title":"Retracted: “Factors Affecting Time to Engraftment During Autologous Stem Cell Transplantation in Patients With Multiple Myeloma”","authors":"","doi":"10.1177/26348535231199808","DOIUrl":"https://doi.org/10.1177/26348535231199808","url":null,"abstract":"","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135650892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535231193889
Ingrid Cristiane Pereira Gomes, João Pedro Costa Machado Teles, Anny Catarina Sousa Coelho, Maria Carollyne Passos Cruz, Lindemberg Costa de Albuquerque, Mariana Amaral Carvalho, Paulo Herlan Castro dos Santos, Sérgio Nolasco dos Santos, Rosana Cipolotti
Lipid alterations have been described in sickle cell anemia (SCA), but their association with the disease severity is not fully understood and their correlation with the nutritional status of this population has not been widely studied. This study aimed to assess the lipid profile and its relation with the nutritional status, including anthropometric characteristics and daily macronutrient intake, and with the severity biomarkers in adults with SCA in a steady state. Cross-sectional study This was an analytical cross-sectional study with 55 adults with SCA and 60 members of the control group. Sociodemographic, anthropometric, nutritional, and laboratory data were collected between March 2019 and June 2020. Mann–Whitney test was used to compare groups and Spearman's correlation coefficient was used to test the relation between variables. The SCA group presented higher calorie, protein, and carbohydrate intake, lower levels of high-density lipoprotein-cholesterol (HDL-c), and a higher triglyceride (TG)/HDL-c ratio than the control. HDL-c showed a positive correlation with hemoglobin and hematocrit (HcT) and a negative correlation with indirect bilirubin and lactate dehydrogenase (LDH). The TG/HDL-c ratio was positively linked with reticulocyte count, LDH, and leukocyte count and negatively linked with Hb. The study findings showed hypocholesterolemia in SCA and its independent correlation with macronutrient intake. The links of HDL-c and TG/HDL-c ratio with hemolysis indices indicate that they are easily accessible, low-cost markers capable of predicting SCA severity.
{"title":"Lipid Profile, Nutritional Status and Severity Biomarkers in Adults With Sickle Cell Anemia","authors":"Ingrid Cristiane Pereira Gomes, João Pedro Costa Machado Teles, Anny Catarina Sousa Coelho, Maria Carollyne Passos Cruz, Lindemberg Costa de Albuquerque, Mariana Amaral Carvalho, Paulo Herlan Castro dos Santos, Sérgio Nolasco dos Santos, Rosana Cipolotti","doi":"10.1177/26348535231193889","DOIUrl":"https://doi.org/10.1177/26348535231193889","url":null,"abstract":"Lipid alterations have been described in sickle cell anemia (SCA), but their association with the disease severity is not fully understood and their correlation with the nutritional status of this population has not been widely studied. This study aimed to assess the lipid profile and its relation with the nutritional status, including anthropometric characteristics and daily macronutrient intake, and with the severity biomarkers in adults with SCA in a steady state. Cross-sectional study This was an analytical cross-sectional study with 55 adults with SCA and 60 members of the control group. Sociodemographic, anthropometric, nutritional, and laboratory data were collected between March 2019 and June 2020. Mann–Whitney test was used to compare groups and Spearman's correlation coefficient was used to test the relation between variables. The SCA group presented higher calorie, protein, and carbohydrate intake, lower levels of high-density lipoprotein-cholesterol (HDL-c), and a higher triglyceride (TG)/HDL-c ratio than the control. HDL-c showed a positive correlation with hemoglobin and hematocrit (HcT) and a negative correlation with indirect bilirubin and lactate dehydrogenase (LDH). The TG/HDL-c ratio was positively linked with reticulocyte count, LDH, and leukocyte count and negatively linked with Hb. The study findings showed hypocholesterolemia in SCA and its independent correlation with macronutrient intake. The links of HDL-c and TG/HDL-c ratio with hemolysis indices indicate that they are easily accessible, low-cost markers capable of predicting SCA severity.","PeriodicalId":29816,"journal":{"name":"Plasmatology","volume":"86 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135650619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.1177/26348535221131252
Jyoti Srivastava, Chad Talton, Pete Vandeberg, Michelle Woznichak, W. Keither Merritt, Marta Jose
Background Hepatitis B (HB) is a worldwide public health problem affecting around 250 million people. Only about 10% of people with HB are aware that they are infected. Vaccination is crucial to prophylactically controlling HB and the combination of vaccination and HB immune globulin (HBIG) are essential in preventing disease after exposure to the HB virus. Objectives In this article, a caprylate-chromatography process has been used for the production of HBIG (HBIG-C). The previously used solvent-detergent process produced an HBIG (HBIG-S/D) with an excellent safety profile but allowed the retention of some procoagulant plasma proteins. The present studies were conducted to assess the character and purity of HBIG produced by the caprylate-chromatography process. Design and Methods Several analytical methods (eg, chromatography, immunoassays, and nephelometry) were used to assess the molecular characteristics, purity and HBIG potency and specific activity of HBIG-C. In addition, testing was conducted to assess the levels of several pro-coagulant factors. HBIG-C was compared with HBIG-S/D and other immunoglobulins manufactured by the S/D process. Results The analysis of HBIG-C showed that the product was almost entirely IgG (99.3 ± 0.2% by electrophoresis) and that the residual IgA was less than that found in S/D products. The IgG present in HBIG-C was 99.7 ± 0.6% monomers and dimers as measured by size exclusion chromatography. Aggregates and fragments constituted < 1%. The IgG subclass distribution in HBIG-C was in the normal reference range. Coagulation factor impurities and pro-coagulant activity were reduced in HBIG-C compared to IgG prepared by the S/D method. Conclusions HBIG-C takes advantage of long-established donor selection processes combined with recently improved manufacturing processes to produce a safe and effective HBIG-product. HBIG-C combines high purity with reduced pro-coagulant factors in a product used for post-exposure prophylaxis of HB.
乙型肝炎(HB)是一个全球性的公共卫生问题，影响着约2.5亿人。只有大约10%的HB患者知道自己被感染了。疫苗接种对于预防控制HB至关重要，疫苗接种和HB免疫球蛋白(HBIG)的结合对于预防暴露于HB病毒后的疾病至关重要。目的采用辛酸色谱法制备HBIG (HBIG- c)。以前使用的溶剂-洗涤剂工艺生产的HBIG (HBIG- s /D)具有良好的安全性，但允许保留一些促凝血浆蛋白。本研究对辛酸色谱法制备的HBIG的性质和纯度进行了评价。设计与方法采用色谱法、免疫分析法、比浊法等多种分析方法评价HBIG- c的分子特性、纯度、HBIG效价和比活性。此外，还进行了测试以评估几种促凝因子的水平。将HBIG-C与HBIG-S/D及S/D法生产的其他免疫球蛋白进行比较。结果HBIG-C分析显示，产物几乎全部为IgG(电泳为99.3±0.2%)，残留IgA少于S/D产物。大小排斥色谱法测定HBIG-C中IgG的单体和二聚体含量为99.7±0.6%。聚集体和碎片构成<1%。HBIG-C中IgG亚类分布在正常参考范围内。与S/D法制备的IgG相比，HBIG-C中的凝血因子杂质和促凝活性降低。结论:HBIG-C利用长期建立的供体选择过程，结合最近改进的制造工艺，生产出安全有效的hbig产品。HBIG-C结合了高纯度和减少的促凝因子，用于乙肝暴露后预防的产品。
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