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Abstarct: Introduction: Therapeutic erythrocytapheresis has some advantages over therapeutic phlebotomy, the standard treatment for cytoreduction in polycythemia and hemochromatosis. Erythrocytapheresis can be performed on different cell separators, each with its own characteristics. We present our experience of therapeutic erythrocytapheresis in the treatment of polycythemia and hemochromatosis with an analysis of the performance of cytoreduction, and a comparison between the characteristics of intermittent- and continuous-flow cell separators. Material and methods: During a 20-year period, 1731 procedures were performed in 125 patients, 1634 (94.4%) with a Haemonetics MCS+ separator and 97 (5.6%) with a Spectra Optia system device. The performance of cytoreduction using the Haemonetics MCS+ separator was analysed in 442 procedures performed in 56 patients and the performance of the two apheresis devices was compared. Results: Haemoglobin (Hb) and haematocrit (Hct) values were significantly reduced after erythrocytapheresis with the Haemonetics MCS+ device (Hb: 18.69%; Hct: 18.73%; p-values both

Introduction: Sickle cell disease (SCD) is an inherited single-gene autosomal recessive disorder. Red cell exchange (RCE) refers to the removal of diseased red blood cells (RBCs) in exchange for healthy donor RBCs. In the present study, RCE was performed in patients with SCD with different crises such as acute chest syndrome, vaso-occlusive crisis, and sequestration crisis. The study was conducted to determine the clinical efficacy of RCE in SCD and to study the clinical profile of the study population. The study will help generate data to support and improve existing knowledge on the clinical efficacy of RCE in SCD. Methods: A prospective observational study of RBC exchange in patients of SCD with the acute crisis was conducted at a tertiary care hospital in South Gujarat including the effect of RCE as a treatment modality in patients of SCD. The study was conducted following guidelines given by the American Society for Apheresis. Results: This study was conducted among 8 patients (7 female and 1 male) with varying age groups from 10 to 29 years. Clinical findings of patients, pulse rate, respiratory rate, and oxygen saturation got improved after the procedure. There was an improvement in PaO2 from 51.63 to 73 after the procedure. There was a decrease in mean sickle hemoglobin (Hb S) level (from 68.452% to 16.438% after procedure) and Hb F level (from 11.938% to 3.063% after procedure) with an increase in Hb A level (from 13.862% to 70.152% after procedure). Conclusion: In the present study, automated RCE procedures in patients with SCD were well tolerated and effective for both acute and chronic complications of SCD in terms of relieving the symptoms by reducing the Hb S level postprocedures.

Background: JAK2‐mutated polycythaemia vera (PV) is associated with reduced survival because of thrombotic events and haematological disease transformation. Therapeutic venesection has traditionally been used to lower haematocrit, but the technique of erythrocytapheresis has emerged over the last decade. Aim: To compare erythrocytapheresis with venesection as treatment for PV by assessing medical efficacy and financial viability. Methods: One hundred sixteen patients with PV who received red cell depletion therapy at Barwon Health between 2014 and 2021 were identified. The haematocrit drop after each session, interval between treatment times and number of sessions required to achieve a haematocrit <0.45 were compared with an independent t test. Thrombosis rates were compared with Pearson's chi‐squared test. Cost‐funding analysis was done by assessing the Weighted Inlier Equivalent Separation and National Weighted Activity Unit funding models. Results: Patients treated with erythrocytapheresis achieved a greater haematocrit drop each treatment session (0.075 vs 0.03, P < 0.01), required fewer sessions to achieve a haematocrit <0.45 (1 vs 4, P < 0.01) and experienced fewer thrombotic complications (8.7% vs 32.1%, P = 0.02) than those treated with venesection. Cost‐funding analysis demonstrated that erythrocytapheresis was more financially viable with a surplus of AU$297 per session compared to a deficit of AU$176 with venesection. Even if funding for venesection is increased, the cost of erythrocytapheresis may be mitigated by a lower number of procedures required per year (3.8 vs 5.3, P < 0.01). Conclusions: Erythrocytapheresis is more efficacious than venesection for the treatment of PV and is accompanied by rapid reductions in haematocrit and reduced thrombotic complications. [ABSTRACT FROM AUTHOR]

Introduction: Sickle cell disease (SCD) is an inherited single-gene autosomal recessive disorder. Red cell exchange (RCE) refers to the removal of diseased red blood cells (RBCs) in exchange for healthy donor RBCs. In the present study, RCE was performed in patients with SCD with different crises such as acute chest syndrome, vaso-occlusive crisis, and sequestration crisis. The study was conducted to determine the clinical efficacy of RCE in SCD and to study the clinical profile of the study population. The study will help generate data to support and improve existing knowledge on the clinical efficacy of RCE in SCD. Methods: A prospective observational study of RBC exchange in patients of SCD with the acute crisis was conducted at a tertiary care hospital in South Gujarat including the effect of RCE as a treatment modality in patients of SCD. The study was conducted following guidelines given by the American Society for Apheresis. Results: This study was conducted among 8 patients (7 female and 1 male) with varying age groups from 10 to 29 years. Clinical findings of patients, pulse rate, respiratory rate, and oxygen saturation got improved after the procedure. There was an improvement in PaO2 from 51.63 to 73 after the procedure. There was a decrease in mean sickle hemoglobin (Hb S) level (from 68.452% to 16.438% after procedure) and Hb F level (from 11.938% to 3.063% after procedure) with an increase in Hb A level (from 13.862% to 70.152% after procedure). Conclusion: In the present study, automated RCE procedures in patients with SCD were well tolerated and effective for both acute and chronic complications of SCD in terms of relieving the symptoms by reducing the Hb S level postprocedures. [ABSTRACT FROM AUTHOR]

The hypercoagulable state associated with sickle cell disease (SCD) can be challenging for apheresis procedures. Among 62 single‐needle red cell exchanges (SN‐RCEs) performed over a 15‐month period, 4 patients experienced 6 hemolytic events with a discolored plasma layer, elevated plasma/RBC interface in the centrifuge, and accompanying alarms of "Cells were detected in plasma line from centrifuge" or "AIM system detected RBC at top of connector." The hemolysis originated from the apheresis instrument because samples from the apheresis belt but not the patients' peripheral blood were positive for hemolysis. Further analysis showed the alarms occurred more often in SN‐RCEs (20.4%) than double‐needle RCEs (2.7%), and the hemolysis was probably secondary to clumping. To optimize SN‐RCE, we increased the anticoagulant dosage by changing Inlet/AC ratio from 13 to 8 and lowered the inlet rate to the level comparable to double‐needle RCE. The adjustments were well‐tolerated with no more hemolysis. [ABSTRACT FROM AUTHOR]

Porphyria cutanea tarda (PCT) is the most common chronic porphyria, with approximate prevalence of 1:10,000. PCT is frequently associated with hepatitis C virus (HCV), malignant lymphoma and iron overload. Here, we present a case of PCT onset subsequent to hepatitis E virus infection (HEV), characterised by symptoms including skin fragility, haemorrhagic bullous skin exanthema, and onycholysis. The patient was successfully treated by erythrocytapheresis and hydroxychloroquine. After exclusion of other possible causes of PCT, HEV infection was identified as the likely trigger of the disease in this genetically predisposed individual, representing the first reported instance of such an association. Erythrocytapheresis emerged as a viable alternative to phlebotomy for PCT treatment. This case underscores the significance of considering HEV infection in the aetiology of PCT and highlights erythrocytapheresis as a promising therapeutic approach [ABSTRACT FROM AUTHOR]

Introduction: Therapeutic erythrocytapheresis has some advantages over therapeutic phlebotomy, the standard treatment for cytoreduction in polycythemia and hemochromatosis. Erythrocytapheresis can be performed on different cell separators, each with its own characteristics. We present our experience of therapeutic erythrocytapheresis in the treatment of polycythemia and hemochromatosis with an analysis of the performance of cytoreduction, and a comparison between the characteristics of intermittent- and continuous-flow cell separators., Material and Methods: During a 20-year period, 1731 procedures were performed in 125 patients, 1634 (94.4%) with a Haemonetics MCS+ separator and 97 (5.6%) with a Spectra Optia system device. The performance of cytoreduction using the Haemonetics MCS+ separator was analysed in 442 procedures performed in 56 patients and the performance of the two apheresis devices was compared., Results: Haemoglobin (Hb) and haematocrit (Hct) values were significantly reduced after erythrocytapheresis with the Haemonetics MCS+ device (Hb: 18.69%; Hct: 18.73%; p-values both <0.001). The reductions of Hb and Hct were significantly higher in the Haemonetics MCS+ procedure (p-value <0.001), but the Spectra Optia procedure depleted a significantly higher RBC volume (495 mL versus 442 mL) in a shorter time (18 min versus 36 min)., Conclusion: Both the Haemonetics MCS+ and Spectra Optia systems proved to be highly efficient and safe in RBC cytoreduction with short procedure times. Erythrocytapheresis reduces the frequency of necessary procedures thereby justifying its therapeutic use especially in eligible patients of working age., Competing Interests: Conflicts of interest The authors have disclosed no conflict of interest., (Copyright © 2024 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.)