Comparison of efficacy of plasma exchange versus intravenous immunoglobulin as an add-on therapy in acute attacks of neuromyelitis optica spectrum disorder.

INTRODUCTION: Plasma exchange (PE) is considered a Category II option for the treatment of acute attacks and relapse cases of neuromyelitis optica spectrum disorder (NMOSD). However, neurologists are also considering intravenous immunoglobulins (IVIg) as an add-on therapy for this disorder. AIMS: The aim of this study is to evaluate the efficacy of PE in acute attacks of NMOSD when compared with IVIg, in terms of improvement in the Expanded disability status scale (EDSS) and activities of daily living (ADL) scale score and levels of anti-Aquaporin P4 (AQP4) antibody in seropositive patients. METHODS: We enrolled 43 NMOSD patients in two groups: Group 1 (n = 29) received steroids and PE, and Group 2 (n = 14) received steroids with IVIg. The baseline EDSS and ADL scores were recorded and compared with scores at the end of therapy, 4 weeks, and 3 months after. Also, anti-AQP4 antibody was measured at baseline and post-therapy in seropositive patients of both groups. RESULTS: We observed a significant difference in EDSS (p = 0.00) and ADL score (p = 0.00) at day 10 and 3 months in both groups. However, no significant difference in EDSS, as well as ADL score from baseline (p = 0.83; p = 0.25) to 3 months (p = 0.85; p = 0.19), was observed when delta change of score at 3 months was compared across the two groups (p = 0.39; p = 0.52). We observed improved visual acuity in both groups with mild improvement in findings of magnetic resonance imaging at 3 months. We observed a significant decline in AQP4 antibody concentration (at day 10) in group 1 seropositive patients (p = 0.013) with improved EDSS (p = 0.027) and ADL scores (p = 0.026) of these patients. CONCLUSIONS: PE should be considered as a choice of an add-on therapy in anti-AQP4 antibody-positive NMOSD patients compared with IVIg as it is more effective in reducing antibody concentrations.

How do I manage a blood product shortage?

BACKGROUND: The demand for blood products sometimes exceeds the available inventory. Blood product inventories are dependent upon the availability of donors, supplies and reagents, and collection staff. During prolonged extreme shortages, blood centers and transfusion services must alter practices to meet the needs of patients. STUDY DESIGN AND METHODS: The Association for the Advancement of Blood and Biotherapies Donor and Blood Component Management Subsection compiled some strategies from its blood center and hospital transfusion service members that could be implemented during blood product shortages. RESULTS: Some strategies that blood centers could use to increase their available inventories include increasing donor recruitment efforts, using alternate types of collection kits, manufacturing low-yield apheresis-derived platelets and/or whole blood-derived platelets, using cold-stored platelets, transferring inventory internally among centers of the same enterprise, using frozen inventory, decreasing standing order quantities, prioritizing allocation to certain patient populations, filling partial orders, and educating customers and blood center staff. Transfusion service strategies that could be implemented to maximize the use of the limited available inventory include increasing patient blood management efforts, using split units, finding alternate blood suppliers, trading blood products with other hospital transfusion services, developing a patient priority list, assembling a hospital committee to decide on triaging priorities, using expired products in extreme situations, and accepting nonconforming products after performing safety checks. DISCUSSION: Blood centers and transfusion services must choose the appropriate strategies to implement based on their needs.

Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) - A case report.

Acquired haemophilia A (AHA) is a rare disorder with mostly idiopathic aetiology that leads to factor VIII (FVIII) deficiency due to coagulation inhibitors formation. Treatment protocol includes immunosuppression and Factor VIII bypassing agents including activated Prothrombin Complex Concentrates (PCC). Nevertheless, the role of plasma exchange is not clear in the treatment of AHA. We report a case of 73 year old male who presented with haematuria, prolonged activated partial thromboplastin time (APTT) and a very high titres of Factor VIII inhibitors of 98 Bethesda units (BU) and was diagnosed with acquired haemophilia A. He failed to respond to multiple immunosuppressive therapies including rituximab. Therefore, therapeutic plasma exchange (TPE) therapy was planned due to persistence of haematuria despite immunosuppressive therapies. After five cycles of plasma exchange, APTT became normal, haematuria subsided and Factor VIII inhibitors became negative. Patient was discharged without any bleeding and in a stable condition. In this index case, plasma exchange played a very crucial role, resulting in recovery of the patient. These results advocate that therapeutic plasma exchange is an effective therapy for acquired haemophilia A.

Role of Plasma Exchange in a Steroid- and IVIG-Refractory Patient with Acute Disseminated Encephalomyelitis: A Case Report.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease usually affecting children and is treated with high-dose steroid therapy. CASE REPORT: An 8-year boy presented with limbs weakness and complete loss of vision and was resistant to steroid therapy. He was further treated with plasma exchange and showed full recovery from the neurological deficit. CONCLUSION: Therapeutic plasma exchange appears to be effective in ADEM patients in reversing the neuropathological process especially refractory to steroids and intravenous immunoglobulin.

Donor lymphocyte infusions: An experience from a tertiary care center of North India.

Donor lymphocyte infusions (DLIs) are often recommended products after allogeneic hematopoietic stem cell transplant to increase graft - versus - leukemia effect. More success rate of DLI has been reported in relapsed posttransplant chronic myeloid leukemia. Whatever the indication for DLI, mortality related to post-DLI infusion is 5%-20%, and more than one-third of patients will develop acute and/or chronic graft versus host disease (GVHD) after DLI. We report two cases where DLIs were used for residual disease after posttransplant. Both of DLI went uneventful. None of the patient's developed signs of GVHD postinfusion. Although both patients expired with different causes, none were related to DLI infusion. Information from published literature suggests that DLI should be administered early after relapse or as a prophylactic strategy in patients receiving T-cell-depleted grafts, and patients with aggressive diseases may benefit from disease reduction before DLI. However, further evidence is required to evaluate its efficacy, especially in relapsed or residual hematological malignancies.

Safety and efficacy of plasmapheresis in treatment of acute fatty liver of pregnancy-a systematic review and meta-analysis.

Introduction: Acute fatty liver of pregnancy (AFLP) is a fatal disease occurring in 3rd trimester. The safety and efficacy of plasmapheresis/plasma exchange (PP/PE) as an adjunctive treatment in patients of AFLP has been studied. We performed systematic review and meta-analysis to estimate the clinical parameters that included mortality rates and improvement of the biochemical parameters including Liver and Renal function enzymes, coagulopathy factors of AFLP patients. Methods: We searched PubMed, Ovid MEDLINE, Cochrane, CINAHL and Scopus, ClinicalTrials.gov. RevMan statistical software was used for meta-analysis. Results: Pooled survival proportion for AFLP patients treated with PP/PE was 87.74% (95% CI: 82.84 to 91.65). Efficacy of PP/PE was studied by its effect on mortality. PE/PP was associated with the reduction in the mortality with pooled odds ratio of 0.51 (95% CI: 0.08 to 3.09) with I2 = 86%. Sensitivity analysis after excluding outlier study, yielded a pooled odds ratio of 0.19 (95% CI: 0.02 to 1.52) with reduced heterogeneity (I2 = 63%). Biochemical parameter analysis demonstrated significant improvement post-PP/PE treatment, including decreased bilirubin (MD: 8.30, 95% CI: 6.75 to 9.84), AST (MD: 107.25, 95% CI: 52.45 to 162.06), ALT (MD: 111.08, 95% CI: 27.18 to 194.97), creatinine (MD: 1.66, 95% CI: 1.39 to 1.93), and Prothrombin time (MD: 5.08, 95% CI: 2.93 to 7.22). Discussion: Despite some heterogeneity, PP/PE shows promise in improving biochemical parameters in AFLP patients. PE can serve as a therapeutic approach for AFLP particularly in severe or refractory cases. PE provides the time for organ to recover and helps in creating a homeostatic environment for liver. Large RCTs and propensity matched studies are needed to better understand the safety and efficacy of the treatment. Systematic review registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022315698.

Review of blood donor deferral with emphasis on donor and patient safety.

INTRODUCTION: Blood donor deferral is a part and parcel of the commitment of the blood transfusion services to assure the safety and health of the blood donor as well as the recipient. Periodic review of blood donor deferral is to incorporate or revoke deferral reasons based on the evidence generated in the process of review. Therefore, emphasis must be laid on preparing strategies to only judiciously defer blood donors, recruit and retain first time blood donors, which needs critical appraisal of the existing deferral policies, so that the evidence based changes can be done. MATERIAL AND METHODS: A retrospective analysis of deferral in blood donors who presented at the blood donation centre of an institute of national importance over a span of nine years (2011-2019). Donors were screened as per the Drugs and Cosmetics Act 1940 and Rules 1945 given by the Ministry of Health and Family Welfare, Govt of India. RESULTS: There were 1,37,935 donors attempts, out of which 20,167 (14.6%) donors were deferred from donating blood. Most of the deferred donors were male (88.5%), first time (86.1%) and temporarily deferred (87.6%). Almost comparable number of donors (49.1 % & 48.5%) were deferred for donor safety and patient safety reasons respectively. Overall the three most common reasons for deferral were low hemoglobin (21.6%), hypertension (11.4%) and history of jaundice (9%). In donor safety reasons, low hemoglobin (43.4%), hypertension (22.9%) and low blood pressure (4.5%), and in patient safety, a history of jaundice (18.6%), common cold (15.8%), and high-risk behavior (8.8%) emerged as the three most common reasons for deferral respectively. CONCLUSION: Blood donor deferral is an essential quality indicator of the blood donor selection process. Initiatives like fortification of dietary ingredients with iron, optimizing protein in diet served in schools under mid-day meal program, screening for iron deficiency, hypertension and education about high-risk behavior in schools and colleges may have long term effects of promotion of better health.

Patient Blood Management and Its Role in Supporting Blood Supply.

Blood donors and voluntary blood donations are essential for ensuring the blood supply that can be maintained by good patient blood management (PBM) practices. This review article explores the role of blood donation in PBM and highlights the importance of donor screening and selection processes in different regions worldwide. The donor health questionnaires and the focused physical examination guidelines have changed in the last decade to increase donor and recipient safety. This article also discusses the status of transfusion practices, including the challenges of ensuring a safe blood supply. Significant among these are the effects of the COVID-19 pandemic on the blood supply chain and the impact of an aging donor population, especially. Promoting autologous donations and other blood conservation strategies are suggested to mitigate these issues. The role of replacement donors and the upper age limit for voluntary blood donation may be decided based on the demography and donor pool. The involvement of C-suite executives is also critical in implementing and running a successful PBM program. The review highlights how these different aspects of blood donation are integral to a successful PBM program and the safety of patients who receive blood transfusions.

Interesting rare case of polyarteritis nodosa related to hepatitis B virus and plasma exchange role? - A case report and review of the literature.

Immune-mediated diseases wherein immune complex-mediated injury is predominant; plasma exchange remains a therapeutic option for vasculitis. Hepatitis B virus-associated polyarteritis nodosa (HBV-PAN) wherein immunosuppressants can be contraindicated, plasma exchanges have a proven role when combined with antiviral therapy. Plasma exchange by hastening the clearance of immune complexes is beneficial in acute organ dysfunction. A 25-year-old male presented with complaints of generalized weakness, tingling numbness and weakness of extremities, joint pain, weight loss, and rashes over arms and legs for 2 months. Hepatitis B workup showed high viral loads of HBV (34 million IU/ml) and hepatitis e antigen positivity (1129.06 U/ml). Cardiac workup showed elevated cardiac enzymes and decreased ejection fraction (40%-45%). The finding of contrast-enhanced computed tomography (CECT) chest and abdomen with CT angiogram abdomen was steady with medium vessel vasculitis. A diagnosis of vasculitis with probable etiology of HBV-related PAN with mononeuritis multiplex and myocarditis was made. He was treated with steroids, tablet tenofovir, and 12 sessions of plasma exchanges. On average, 2078 ml of plasma was exchanged during each session with 4% albumin as a replacement fluid using central femoral line dialysis catheter as vascular access on automated cell separator Optia ®Spectra (Terumo BCT, Lakewood, Co). He was discharged with the resolution of symptoms, including myocarditis and increase in power strength and still in follow-up. The present index case indicates that antiviral combined with plasma exchange after short-term corticosteroids is an effective therapy for HBV-PAN. TPE can be used as adjuvant therapy along with antiviral therapy in a rare disease like HBV-related PAN.

Effectiveness of therapeutic plasma exchange in case of rare neurological disorder Isaacs syndrome.

Isaacs syndrome is a disease characterized by nerve hyperexcitability and pseudomyotonia and treated with immunomodulatory and symptomatic therapy approaches. Here, we report a case of anti-(leucine-rich glioma-inactivated 1) antibody-positive patient diagnosed as Isaacs syndrome and accomplished a nearly complete response to only four sessions of therapeutic plasma exchange (TPE). Our experience suggests that TPE along with other immunomodulatory agents may be beneficial and well-tolerated approach in patient with Isaacs syndrome.

Exploration of COVID-19 related fears deterring from blood donation in India.

INTRODUCTION: The coronavirus pandemic (COVID-19) has impacted and pushed the healthcare settings to extremes across the globe. It was extremely challenging to sustain blood donation, and strategies could be formulated on knowing fears hindering blood donation. METHODS: A cross-sectional survey using Google Forms® through WhatsApp and email after obtaining the ethical clearance. The survey questionnaire was validated for content using the Delphi technique, and pilot tested for finalization. RESULTS: The survey was attempted by 1066 participants, and 749 participants who had not donated since pandemic were included in the study. A little more than half, 415 (55%) reported either one or more than one fear during the pandemic which hindered blood donation. They reported lack of confidence in the safety measures at the hospitals and fear of transmitting infection to family, in 415 (55%) of the participants each, respectively. The fear of COVID-19 hospital infection risk and hospital entry was statistically significant across the age groups that are eligible for blood donation. CONCLUSIONS: The clear and dedicated confidence building measures to sustain blood donation using all communication modalities clearly emerge as the most important strategies to augment blood donation in the COVID-19 pandemic. The measures should include information about implementation of safety measures to mitigate COVID-19 transmission at the blood centres and that the act of blood donation does not increase risk of COVID-19 and therefore the risk of transmission of infection to family.

Centrifugal Therapeutic Plasma Exchange in Pediatric Patients.

OBJECTIVE: To assess the safety of centrifugal therapeutic plasma exchange (TPE) in pediatric patients. METHODS: The authors did a retrospective analysis of all TPE procedures performed in pediatric patients over a period of 19 y (2001-2019). Procedures were done on different apheretic devices, daily or on alternate days depending on the clinical condition of the patient. Adverse events during the procedure were noted and analyzed. RESULTS: A total of 672 TPE (with mean of 6.77 ± 4.85) procedures were performed for 99 pediatric patients with different indications like hematological (n = 68), renal (n = 12), neurology (n = 18) and hepatology (n = 1). The mean age was 7.00 ± 3.11 y and weight was 20.72 ± 9.17 kg. Adverse events (AEs) were observed during 34 (5%) procedures, most common were allergic reactions to replacement fluid (2.24%) followed by hypotension (1.04%), symptomatic hypocalcemia (1.04%), line occlusion (0.59%), and febrile non hemolytic transfusion reaction (0.41%). A significant correlation of AEs was observed with weight (p = 0.045), total blood volume of the patient (p = 0.04), increasing number of procedures (p = 0.000) and replacement fluid [Fresh frozen plasma (FFP)] (p = 0.04). All AEs were managed as per departmental standard operating procedures (SOPs) completing procedures successfully except one which was abandoned. No mortality was observed during the procedures. CONCLUSION: TPE is a safe therapeutic modality in pediatric patients when performed under expert technical supervision with proper SOPs in place.

Role of plasma exchange in postpartum microangiopathies: An experience from a tertiary care center.

BACKGROUND: Postpartum microangiopathies are rare but are associated with high maternal and fetal mortality requiring early diagnosis and prompt treatment to improve the outcome. AIMS AND OBJECTIVES: This retrospective study aims to assess the efficacy of plasma exchange (PE) therapy in postpartum thrombotic microangiopathies. MATERIALS AND METHODS: We did retrospective analysis of all plasma exchange procedures performed in patients of postpartum thrombotic microangiopathies over a period of 1 year (2015-2016). Patient's pre- and post-plasma exchange hematological and biochemical parameters were recorded and compared for analyzing the response to the therapy. Patients were followed telephonically even after their discharge from the hospital. RESULTS: Hematological and renal profile improved in 8 out of 9 patients after PE therapy. Survival after PE therapy was 40% in post partum atypical HUS and 75% in patients with HELLP syndrome at 4 months of follow up. CONCLUSION: Early initiation of PE therapy in postpartum thrombotic microangiopathies can reduce morbidity and mortality associated with them.

Comparative evaluation of enzyme-linked immunosorbent assay with rapid plasma reagin for screening of syphilis in blood donors.

World Health Organization (WHO) recommends screening of syphilis in low prevalence populations of blood donors by treponemal tests like enzyme-linked immunosorbent assay (ELISA), whereas in India screening is done by rapid plasma reagin (RPR). The present pilot study evaluated the performance of ELISA compared to RPR, keeping Treponema pallidum hemagglutination assay as a reference test. ELISA was equally sensitive (100%), more specific (56.3% vs. 0%), more accurate (83.7% vs. 62.7%), had better positive predictive value (79.4% vs. 62.8%) and negative predictive value (100% vs. 0%), and less biological false positivity (37.2% vs. 20.6%) when compared to RPR. The WHO recommendations of screening for syphilis in low prevalence population of blood donors using ELISA may be adopted for usage in transfusion services that have the facility of ELISA.

First report of the impact on voluntary blood donation by the blood mobile from India.

INTRODUCTION: The blood mobile is one of the modern methods of mobile blood collection facility funded through the third phase of National AIDS Control Programme by the National Blood Transfusion Council of India. MATERIAL AND METHODS: A retrospective analysis of data in relation to the blood mobile was carried out with respect to the number of blood donation camps, number of blood units collected, adverse donor reactions, and the expenditure that occurred during the blood collection in the blood mobile from 1(st) January 2012 to 30(th) June 2014. RESULTS: There were 64, 84 and 62 blood donation camps conducted in the blood mobile with collection of 3301, 5166 and 2842 blood units during 2012, 2013 and the first half of 2014. The percentage of voluntary blood collection in blood mobile was 8.5% in 2012, increased to 12.4% in 2013 and stands at 14.39% in the first half of 2014. The difference in the means of the adverse donor reactions in the blood mobile and the outdoor camps was not statistically significant. DISCUSSION AND CONCLUSION: The blood mobile is definitely an asset as far as augmentation of voluntary blood donation is concerned, ensures stable collection of blood for better provision of blood and blood components. However the facility requires a comprehensive annual maintenance with incorporation of onsite quick response team both from the manufacturer of the vehicle, and the blood collection equipments. Adequate provision of funding for operational expenditure would in turn facilitate optimum utilization of this facility.