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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.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.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.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.

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.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.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.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.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.

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.

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.To assess the efficacy of TPE in symptomatic patients of NMOSD not responding to high dose intravenous steroids.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.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.The addition of TPE as a part of therapy is effective and safe in acute exacerbations of NMOSD.

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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.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.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).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.

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.

ObjectivesConvalescent plasma (CP) as a passive source of neutralizing antibodies and immunomodulators is a century-old therapeutic option used for the management of viral diseases. We investigated its effectiveness for the treatment of COVID-19.DesignOpen-label, parallel-arm, phase II, multicentre, randomized controlled trial.SettingThirty-nine public and private hospitals across India.ParticipantsHospitalized, moderately ill confirmed COVID-19 patients (PaO2/FiO2: 200-300 or respiratory rate > 24/min and SpO2 ≤ 93% on room air).InterventionParticipants were randomized to either control (best standard of care (BSC)) or intervention (CP + BSC) arm. Two doses of 200 mL CP was transfused 24 hours apart in the intervention arm.Main Outcome MeasureComposite of progression to severe disease (PaO2/FiO2< 100) or all-cause mortality at 28 days post-enrolment.ResultsBetween 22ndApril to 14thJuly 2020, 464 participants were enrolled; 235 and 229 in intervention and control arm, respectively. Composite primary outcome was achieved in 44 (18.7%) participants in the intervention arm and 41 (17.9%) in the control arm [aOR: 1.09; 95% CI: 0.67, 1.77]. Mortality was documented in 34 (13.6%) and 31 (14.6%) participants in intervention and control arm, respectively [aOR) 1.06 95% CI: −0.61 to 1.83].InterpretationCP was not associated with reduction in mortality or progression to severe COVID-19. This trial has high generalizability and approximates real-life setting of CP therapy in settings with limited laboratory capacity. A priori measurement of neutralizing antibody titres in donors and participants may further clarify the role of CP in management of COVID-19.Trial registrationThe trial was registered with Clinical Trial Registry of India (CTRI); CTRI/2020/04/024775.

BACKGROUND: Thrombopoietin (TPO) is regulated by a feedback mechanism between megakaryocytes and platelets. This is important in plateletpheresis donors to compensate for donation-associated platelet loss. AIMS AND OBJECTIVES: The aim and objective of this study were to investigate changes in serum TPO levels in healthy plateletpheresis donors and its correlation with platelet recovery pattern. MATERIALS AND METHODS: Out of 50 plateletpheresis donors recruited in the study over 1 year, only 29 completed follow-up and were further analyzed. Plateletpheresis procedures were performed on two types of cell separators (TRIMA ACCEL®, Terumo BCT Lakewood Colorado and AMICUS®, Fresenius Kabi, Germany). Platelet parameters were estimated pre- and post-platelet donation, at 3rd- and 5th-day postdonation. Serum TPO levels were determined using quantitative sandwich enzyme-linked immunosorbent assay technique (Raybiotech, USA) as per the protocol of the manufacturer. RESULTS: The majority of donors (72%) in our study were first-time donors. The baseline platelet count was 226 ± 44 × 103/μl with a significant decline (30%; P < 0.001) in postdonation phase and remained below baseline on the 3rd and 5th day. The serum TPO levels increased significantly (P < 0.001) from a baseline of 227.81 (interquartile range [IQR]: 176.06) pg/ml to 269.94 (IQR: 110.68) pg/ml postdonation and remained elevated from baseline levels on the 3rd and 5th day. An inverse relation was observed between change in serum TPO levels and platelet count during postdonation phase which was not statistically significant (P > 0.05). CONCLUSION: Serum TPO levels increase significantly post plateletpheresis donation corresponding to decrease in platelet counts showing that TPO plays a vital role in compensatory mechanism after platelet loss.

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.

Background The majority of patients in low- and middle-income countries (LMIC) are unable to receive optimal therapy, including autologous stem cell transplant (ASCT) for high-risk neuroblastoma. Management is intensive and multidisciplinary; survival is often poor. We report a single-center outcome of high-risk neuroblastoma, with adaptations optimized for LMIC. Procedure The study was retrospective. Patients were treated on the backbone of the high-risk neuroblastoma study-1 of SIOP-Europe (HR-NBL1/SIOPEN) protocol with ASCT. Adaptations incorporated to decrease cost, requirement for inpatient admission, infections, and faster engraftment included (a) optional outpatient administration for rapid-COJEC, (b) two sessions of stem-cell apheresis, (c) storing stem cells at 2-6°C without cryopreservation for up to 7 days, (d) no central lines, (e) no antibacterial/antifungal/antiviral prophylaxis, (f) omitting formal assessment of cardiac/renal/pulmonary functions before ASCT, and (g) administration of pegylated granulocyte colony-stimulating factor on Day +4. Results Over 5 years 9 months, 35 patients with high-risk neuroblastoma were treated. Rapid-COJEC was administered over a median duration of 80 days (interquartile range: 77, 83). Conditioning regimen included melphalan (n = 7), oral busulfan-melphalan (Bu/Mel; n = 6), or intravenous Bu/Mel (n = 22). The median viability of stem cells stored for 6 days (n = 28) was 93% (range: 88-99). Two (5.7%) patients had ASCT-related mortality. The 3-year overall and event-free survival was 41% and 39%, respectively. A relapse occurred in 20 (57%) patients. Treatment abandonment was observed in one (3%) patient. Conclusions Administration of therapy in a disciplined time frame along with low-cost adaptations enables to manage high-risk neuroblastoma with low abandonment and an encouraging survival in LMIC. Stem cells can be stored safely without cryopreservation for up to 7 days.

Background Therapeutic Plasma Exchange (TPE) and Intravenous Immunoglobulin both are first-line treatments for Guillain Barre Syndrome; however, there is a significant difference in cost. We undertook this study to assess the cost minimization for treating Guillain Barre Syndrome patients. Methods A prospective randomized controlled trial was undertaken, in which 40 Guillain Barre Syndrome (GBS) patients with a GBS disability score of grade four and five were enrolled. A societal perspective was adopted for the analysis and assessment of both the health system cost and out-of-pocket expenditures. Cost-minimization analysis was undertaken as both the treatments were equally effective at the end of 12 weeks. Results No statistically significant differences were observed in the GBS Disability scores during overall treatment course in both treatment groups. The Out-of-pocket cost for the immunoglobulin (IVIG) group was INR 219 247 (4298 USD) and for the TPE group was INR 104 070 (2040.5 USD). Overall INR 86 685 ($1700), that is, 53% higher cost was observed in IVIG group without any concomitant health outcome benefit. Conclusion In comparison with IVIG, TPE appears to be the better option for treatment of GBS in cost-constraint countries like ours to provide an economic treatment option to most average people.

Febrile neutropenia is a common complication of chemotherapy especially in hematological malignancies associated with sepsis or severe infection. We report a case where a seven-year-old girl with T – cell acute lymphoblastic leukemia (ALL) developed febrile neutropenia (absolute neutrophil count - ANC

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.

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.

Background Therapeutic whole blood exchange (TWBE) has been used as an alternative when methylene blue (MB) fails in severe methaemoglobinemia. However, there are limited data on the efficacy and safety of TWBE. Objectives Our aim was to report our institutional experience with TWBE. We also perform a systematic review of published literature. Methods We retrospectively reviewed our respiratory intensive care unit database to identify cases of methaemoglobinemia managed with TWBE. A systematic review of the PubMed database was performed to identify similar cases (≥12 years). We report the indications, utility, and safety of therapeutic exchange in methaemoglobinemia. The procedural details were also noted. Results We identified five subjects who received TWBE for methaemoglobinemia (median methaemoglobin level 39%; range 19.6-42.4%). TWBE was successful in all five cases and no adverse events were encountered. Our review identified 27 additional subjects. The median methaemoglobin level was 37.5% (range 3.7-81%). The most common indication (n = 24, 75%) for therapeutic exchange was a lack of response to MB. A majority of the subjects (n = 26/32, 81.2%) survived. No procedure-related complications were reported. Conclusion TWBE is a safe and effective salvage modality for adults with methaemoglobinemia, when MB is either contraindicated or ineffective. Future studies should standardise therapeutic exchange in the management of methaemoglobinemia.

Background & objectives: Individual donation nucleic acid testing (ID-NAT) is considered as sensitive technology to assess blood safety from viral transfusion-transmissible infections (TTIs) in blood donors. The present study was aimed to analyze the results of ID-NAT for three years (2013-2015) with special reference to different types of donors and their age ranges in a tertiary care centre in north India. Methods: The results of ID-NAT for three years were retrospectively analyzed at our centre. A total of 168,433 donations were tested with ID-NAT, of which 10,467 were tested with Procleix® Ultrio® reagents and 157,966 were tested with Procleix®UltrioPlus® reagents, and the results were compared with those of serology to calculate the NAT yield in voluntary, replacement, first-time and repeat donors. Results: A combined NAT yield was observed as one in 1031 out of 167,069 seronegative donations with HBV yield as one in 1465, HCV yield as one in 3885 and HIV-1 as one in 167,069. Yield for co-infection (HCV and HBV) was one in 41,767. A high NAT yield was observed in replacement donors (1 in 498) as compared to voluntary donors (1 in 1320). Interpretation & conclusions: Addition of NAT to serology improved the blood safety in our centre interdicting possibility of 150 TTIs annually. It has also reemphasized the safety of voluntary over replacement donors. The results also highlight the need of proper counselling, notification and referral guidelines of NAT yield donors in our country and other countries which lack them.

To assess the safety of centrifugal therapeutic plasma exchange (TPE) in pediatric patients. 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. 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. TPE is a safe therapeutic modality in pediatric patients when performed under expert technical supervision with proper SOPs in place.

Platelet refractoriness, which leads to platelet transfusion failure resulting in significant morbidity and long hospital stay, is routinely not investigated.To determine the efficacy of cross-match compatible platelets in multi-transfused alloimmunized hemato-oncological patients refractory to platelet transfusion.149 ABO compatible single donor apheresis platelet transfusions given to 38 alloimmunized refractory patients. Corrected Count Increment (CCI)5000 (1 h) was taken to define refractoriness. Solid-phase red cell adherence assay was used to determine the alloimmunization status and platelet cross-matching. Post Transfusion Platelet Increment, CCI and the Percentage Platelet Recovery were used to monitor the effectiveness of platelet transfusion. ANOVA test followed by Post hoc test Tukey HSD used to compare group means and classified into three groups depending upon the cross-matching and compatibility status. Categorical data was analysed for various outcomes using Pearson's chi square test or Fischer exact test.Patients showed statistically significant recovery in terms of PPI, CCI and PPR at 1 h post SDAP transfusions when they received cross-matched compatible platelets. The one-hour CCI was significantly higher for cross-match-compatible platelets (19173 ± 2692) than for incompatible (5888 ± 1526) and for uncross-matched (8140 ± 1480). Forty four (97.8%) of 45 cross-matched compatible platelet transfusion episodes showed a satisfactory response in terms of PPI and CCI values as compared to 50 % and 53.9% in uncross-matched group respectively (p 0.0001).Platelet cross-matching is an effective intervention in the management of multi-transfused alloimmunized Haemato-oncological patients, refractory to platelet transfusion.

Introduction To determine an optimal platelet dose in thrombocytopenic patients is important for their judicious use. Transfusing platelets in different doses and comparing their post transfusion response can achieve this. Aim To compare the efficacy of low and high dose single donor apheresis platelets (SDAP) with standard dose transfusions in terms of Corrected Count Increment (CCI), Percent Platelet Recovery (PPR) and transfusion free interval. Method It was a prospective case control study done from January 2016 to April 2017. Twenty-eight hemato-oncology patients with CCI ≥5000 at 20–24 hours after standard dose (3 × 1011/unit), received low dose (1.5 × 1011 platelets/unit) and high dose (>4 × 1011 platelets/unit) SDAP. CCI and PPR were calculated after 20 to 24 hours of transfusion. Transfusion free interval and bleeding episodes were also noted. Grading was done according to WHO bleeding scale. Result There was no statistical difference in CCI and PPR when standard dose was compared with low dose (CCI: p = 0.92, PPR: p = 0.89). When standard and high dose was compared, standard dose gave better results than the high dose in terms of CCI (p = 0.006) and PPR (p = 0.008) although the post transfusion increments were comparable (p = 0.938). High dose gave better (p = 0.005) platelet count increments than low dose but CCI (p = 0.04) and PPR (p = 0.05) was significantly less than the low dose. The difference in transfusion free intervals after three doses was not significant. Donor exposure to the patients was significantly (p = 0.000) reduced to 17.5%. Conclusion Possibility of low dose as an alternative to standard dose can be considered in view of comparable platelet response indicators and significantly reduced donor exposure.

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.

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 ± 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.

Background: Therapeutic plasma exchange (TPE) in atypical hemolytic uremic syndrome (aHUS) is considered as first line treatment as per current American Society for Apheresis (ASFA) guidelines. But there is very limited data available in the literature regarding efficacy and safety of TPE procedures in pediatric aHUS patients. Aim: To assess the safety and efficacy of TPE by using apheresis devices in pediatric aHUS patients. Materials and Methods: We did a retrospective analysis of all TPE procedures performed in aHUS pediatric patients over a period of 13 years (2001–2013). TPE procedures were done on two different devices daily or on alternate days depending on clinical condition of the patient. Adverse events if any were noted and analyzed. Pre and post procedural laboratory profiles were analyzed to assess the response to TPE therapy and patients were categorized accordingly. Results: A total of 169 TPE procedures (range of 1–22/patient with an average of 7.6 procedures/patient) were performed on 30 pediatric patients. Twenty four patients had more than 3 TPE procedures. Sixteen patients were complete responders, 5 were partial responders and 3 were non responders. The time between onset of illness and start of TPE therapy was 1–4 days in complete responders, 5–7 days in partial responders and 8–9 days in non- responders. Adverse events were observed in 13 (7.7%) procedures. Conclusion: TPE is a safe and effective therapeutic modality in pediatric aHUS if instituted early in the course of disease with a minimum of four to five procedures. J. Clin. Apheresis, 2015. © 2015 Wiley Periodicals, Inc.

Therapeutic plasma exchange is established treatment for patients with thrombotic thrombocytopenic purpura (TTP) and for refractory cases immunosuppressive therapy can also be considered. We present a refractory case of TTP which was managed with therapeutic plasma exchange and rituximab.

Acute exacerbations and complications are common in patients of systemic lupus erythematosus (SLE) despite of adequate long-term therapy with immunosuppressive drugs. So other options like therapeutic plasma exchange (TPE) can be considered as part of management. Here, we share our experience of two patients of SLE with acute complications who were successfully managed with TPE.

Therapeutic plasma exchange (TPE) in atypical hemolytic uremic syndrome (aHUS) is considered as first line treatment as per current American Society for Apheresis (ASFA) guidelines. But there is very limited data available in the literature regarding efficacy and safety of TPE procedures in pediatric aHUS patients.To assess the safety and efficacy of TPE by using apheresis devices in pediatric aHUS patients.We did a retrospective analysis of all TPE procedures performed in aHUS pediatric patients over a period of 13 years (2001-2013). TPE procedures were done on two different devices daily or on alternate days depending on clinical condition of the patient. Adverse events if any were noted and analyzed. Pre and post procedural laboratory profiles were analyzed to assess the response to TPE therapy and patients were categorized accordingly.A total of 169 TPE procedures (range of 1-22/patient with an average of 7.6 procedures/patient) were performed on 30 pediatric patients. Twenty four patients had more than 3 TPE procedures. Sixteen patients were complete responders, 5 were partial responders and 3 were non responders. The time between onset of illness and start of TPE therapy was 1-4 days in complete responders, 5-7 days in partial responders and 8-9 days in non- responders. Adverse events were observed in 13 (7.7%) procedures.TPE is a safe and effective therapeutic modality in pediatric aHUS if instituted early in the course of disease with a minimum of four to five procedures. J. Clin. Apheresis 31:381-387, 2016. © 2015 Wiley Periodicals, Inc.

Background: Dengue fever (DF) going into dengue shock syndrome (DSS) and dengue hemorrhagic fever (DHF) has challenged the blood transfusion services in the form of urgent requirement of platelets. Single donor apheresis platelets technology has helped the transfusion services that would have otherwise to arrange a large inventory of random donor platelets. Aim: To analyze the critical issues in the management of dengue outbreak during the year 2012 at a tertiary care institute. Study Design and Method: This is a retrospective study of records of the 186 serology proven dengue patients' requisitions, who received SDAP transfusion support from our department and analyses of the critical issues faced in managing the dengue patients. Results: A total of 239 single donor apheresis platelets (SDAP) procedures were done for 186 patients with serology proven dengue infection. Total 139(58.15%) SDAP requisitions were received from our own institution while 100(41.84%) requisitions were from other hospitals in the region. We observed that 20.43% SDAP requisitions did not qualify either prophylactic or therapeutic criteria for platelet transfusion according to the current guidelines. A total of 205 voluntary SDAP donors are registered in our emergency donor panel for plateletpheresis, out of this only 29 (14.14%) responded for SDAP donation during the dengue outbreak. Conclusions: Following appropriate guidelines for prophylactic and therapeutic blood and blood components transfusions and a well-planned emergency donor panel registry are essential pre- requisites for successful