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.

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.

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.

Severe copper sulphate poisoning: A case rescued with plasmapheresis.

Acute copper sulphate poisoning is associated with multi-organ failure and high mortality. Patients typically present with gastrointestinal symptoms, haemolysis, methaemoglobinaemia, acute liver injury, rhabdomyolysis and renal failure. Management is usually supportive, and the role of chelation therapy has not been established. Copper is not dialysable. Plasmapheresis has been shown to remove protein-bound copper, reducing plasma and intracellular concentrations. We present a case of severe copper sulphate poisoning, who did not improve with chelation therapy with D-penicillamine and supportive care, but with therapeutic plasma exchange (four cycles) showed rapid clinical recovery.

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.

Serial analysis of hematological, biochemical, and immunological parameters alterations in regular healthy voluntary donors during plateletpheresis donation.

BACKGROUND AND OBJECTIVES: The long-term effect of regular plateletpheresis on donors has not been characterized. Hence, we planned to study the long-term alterations in hematological, biochemical, and immunological parameters in regular repeat platelet apheresis donors. MATERIALS AND METHODS: Thirty-three healthy voluntary regular repeat apheresis donors presenting for platelet donation, fulfilling the requisite donor selection criteria, underwent sequential analysis of the hematological, biochemical, and immunological parameters over 1 year. RESULTS: A total of 33 regular repeat donors were enrolled in the study; out of these, 22 could be followed up to 3 months, 12 up to 6 months, and 10 donors up to 12 months for their hematological, biochemical, and immunological parameters. Overall, there was no significant change in hematological profile except a rise in platelet count at 3 months (P = 0.023) with no significant difference at 6 and 12 months from the baseline. In addition, serum thrombopoietin levels at 3 months (P = 0.010) and serum erythropoietin at 6 months (P = 0.01) were significantly higher than baseline. Mean platelet volume was significantly higher from baseline at 12 months (P = 0.00). Serum protein, lymphocyte subpopulation, and serum ferritin did not show any significant change from baseline over 12 months of follow-up. However, there was a significant decline (P = 0.00) in serum calcium and an increase in serum magnesium from baseline (P = 0.03) at 12 months. INTERPRETATIONS AND CONCLUSIONS: To conclude, apheresis platelet donation is a safe procedure. However, a complete hematological, biochemical, immunological profile and bone marrow density at regular intervals (3-6 months) are recommended to ensure the safety of regular repeat plateletpheresis donors.

Therapeutic plasma exchange is a safe and effective bridge therapy in patients with alcohol-associated ACLF not having immediate prospects for liver transplantation-A case-control, pilot study.

BACKGROUND: Therapeutic plasma exchange (TPE) is a well-established treatment modality in acute liver failure patients, but its efficacy in treating acute on chronic liver failure (ACLF) patients is yet to be established. AIM: To assess the efficacy and safety of TPE in patients with alcohol-associated ACLF who were nonresponders to standard medical treatment (SMT) and without immediate prospects for liver transplantation. METHODS: Twenty-eight alcohol-related ACLF (grade II) patients (14 cases and 14 controls) were enrolled in the study. Cases underwent standard volume TPE along with SMT while the controls were on SMT alone. The change (baseline to day 10) in laboratory parameters, cytokine concentrations, clinical severity scores along with 30 and 90 day mortality rates were noted and compared between the two groups. The adverse events (AEs) were noted in the groups and analyzed. RESULTS: A total of 51 TPE procedures were performed in 14 patients (average of 3.62 procedures/patient). TPE was effective in reduction of serum bilirubin, ammonia, activated partial thromboplastin time, prothrombin time, international normalized ratio, and severity scores (ACLF Research Consortium, Maddrey's discriminant function, and model for end-stage liver disease) (P < .05). There was no significant difference in the reduction of serum interleukin-6 (IL-6), IL-10, and tumor necrosis factor-α concentrations among cases. Among the cases who received the complete TPE interventions, 30- and 90-day mortality rates were lower in the cases as compared to controls albeit only the 90-day mortality was significantly different. Procedure-related AEs was observed in 2% of procedures. CONCLUSION: TPE is an effective and well-tolerated bridge therapy in patients with alcohol-associated ACLF of moderate severity not improving on SMT and without immediate prospects for liver transplantation.

Therapeutic Plasma Exchange in Neuromyelitis Optica Spectrum Disorders-Experience from Tertiary Care Center in North India.

Background: Therapeutic plasma exchange (TPE) is indicated in the acute presentation of neuromyelitis optica spectrum disorders (NMOSD) as category II indication as per American Society for Apheresis guidelines 2016. Aim: To assess the efficacy of TPE in symptomatic patients of NMOSD not responding to high dose intravenous steroids. Patients and Methods: We retrospectively reviewed the record of TPE procedures in NMOSD patients over a period of 3 years (2013-2016). TPEs were done on Cobe® spectra (Terumo BCT, Lakewood Co., USA) using 5% human serum albumin or fresh frozen plasma as replacement fluid. Clinical improvement was recorded post-procedure and adverse reactions if any was noted. Results: We performed TPE in 11 clinically diagnosed patients of NMOSD. Three (27%) patients had only visual symptoms, five (46%) had both visual as well as muscular symptoms, whereas three (27%) patients presented with only muscular symptoms. Out of seven tested, three patients were aquaporin-4-immunoglobulin-G-positive (AQP4-IgG positive). Visual symptoms improved from no vision pre-TPE therapy to finger counting or 6/24 post-therapy.The muscle power of the limbs improved by grade one to grade two post-therapy. Adverse events were observed in 8% (5/62) of the procedures; allergic reactions to replacement fluid as the most common event (n = 3) followed by hypotension (n = 2). Follow-up was available in 81% (9/11) patients. Two patients died at 3 and 12 months of follow-up. One patient had relapsed and underwent a second TPE cycle. Conclusion: The addition of TPE as a part of therapy is effective and safe in acute exacerbations of NMOSD.

Report of plasma exchange in a rare case of association of myasthenia gravis with thymoma and parathyroid adenoma.

Myasthenia with thymoma and parathyroid adenoma is a rare presentation. Very few cases have been reported of this association without much role of plasma exchange in these patients. Here, we present our experience of plasma exchange in this rare clinical entity.

Anti-Compliment Factor H Antibody Associated Hemolytic Uremic Syndrome in Children with Abbreviated Plasma Exchanges: A 12-Month Follow-up Study.

INTRODUCTION: Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria. METHODS: A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS. Follow up details including physical examination, hematological parameters, renal function test and urine examination performed at 3, 6, and 12 months were noted. Risk stratification and staging for chronic kidney disease (CKD) were done according to the Kidney Disease Improving Global Outcomes (KDOQI) guidelines. Standard statistical tests which were appropriate were used. RESULTS: Mean age of our study cohort was 7.8 ± 1.9 years. 14 children had hypertension. Mean nadir hemoglobin was 5.8 ± 1.0 g/dL and platelet = 58 ± 37.7 × 109 cells/L. Median anti factor H (AFH) level was 316 AU/mL (150 to 452). Hemodialysis was required in 7 children. Fourteen children received PLEX with a mean of 11 ± 6 cycles. Thirteen children received 6 cycles of intravenous cyclophosphamide. After six months, therapy was switched to mycophenolate mofetil in 4 children, steroids alone in 2 children and 9 children with azathioprine. On follow-up, risk of CKD reduced from 80% at 3 months to 26% at 12 months (P = .01). Only 40% patients had CKD stage 2 at the end of 12 months (mean eGFR = 95.0 ± 19.4 mL/min/1.73m2). CONCLUSION: The adequate number of PLEX needed in AFH-HUS needs further studies. Till such reports come, PLEX in recommended strategies or lesser, if not available, with immunosuppression in AFH-HUS can decrease progression to CKD. DOI: 10.52547/ijkd.6507.

Therapeutic Plasma Exchange: A Lifesaving Therapy in Case of ANCA-associated Vasculitis with Diffuse Alveolar Hemorrhage.

How to cite this article: Tripathi PP, Sharma RR, Chhabria B, Hans R, Sehgal IS. Therapeutic Plasma Exchange: A Lifesaving Therapy in Case of ANCA-associated Vasculitis with Diffuse Alveolar Hemorrhage. Indian J Crit Care Med 2021;25(7):828-829.

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.

Use of convalescent plasma for COVID-19 in India: A review & practical guidelines.

Convalescent plasma (CP) therapy is one of the promising therapies being tried for COVID-19 patients. This passive immunity mode involves separating preformed antibodies against SARS-CoV-2 from a recently recovered COVID-19 patient and infusing it into a patient with active disease or an exposed individual for prophylaxis. Its advantages include ease of production, rapid deployment, specificity against the target infectious agent, and scalability. In the current pandemic, it has been used on a large scale across the globe and also in India. However, unequivocal proof of efficacy and effectiveness in COVID-19 is still not available. Various CP therapy parameters such as donor selection, antibody quantification, timing of use, and dosing need to be considered before its use. The current review attempts to summarize the available evidence and provide recommendations for setting up CP protocols in clinical and research settings.

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 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.

Anti-complement factor I antibody associated atypical hemolytic uremic syndrome - A new insight for future perspective!

Atypical hemolytic uremic syndrome (aHUS) is caused mainly by complement dysregulation. Although various defects in the complement system explaining pathophysiology have been described in recent years, the etiology still remains unclear in about thirty percent of cases. In exploring other causes, similar to anti- complement factor H (anti-CFH) antibody associated HUS, we hypothesized that anti-complement factor I (anti-CFI) antibody could play a role in aHUS. Further, we tried to describe the clinical profile and outcome of those with high anti CFI antibody titers. Eleven of thirty five children (31 %) diagnosed with aHUS from July 2017 to December 2018 had high IgG anti-CFI antibody titers. Median age was 10 months (6, 33) with no sex difference. Thirty-six percent (4/11) had nephrotic-range proteinuria. C3 was low in 8 children (72.7 %) with mean C3 (68.1 ±â€¯14.7 mg/dL). Plasmapheresis was done in 2 children who promptly responded, suggesting the possible role of anti-CFI antibody in pathogenesis of aHUS in these patients. Further studies examining role of anti-CFI antibodies in aHUS is warranted with longitudinal and genetic studies.