A comparison between erythrocytapheresis and venesection for the treatment of JAK2-mutated polycythaemia.

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.

Eritroaféresis terapéutica: experiencia en pacientes con policitemia vera y eritrocitosis secundaria / Therapeutic erythroapheresis: Experience in patients with polycythemia vera and secondary erythrocytosis

La eritrocitaféresis terapéutica (ET) es una estrategia más eficiente que la flebotomía en la reducción del hematocrito en las eritrocitosis primarias y secundarias. Objetivo: Analizar la tasa de respuesta y seguridad de la ET en policitemia vera (PV) y eritrocitosis secundaria (ES). Pacientes y método: Revisión retrospectiva de los pacientes con PV o ES tratados con ET, ante el fracaso a flebotomías o con comorbilidades que impedían cambios importantes de volemia. Resultados: Se realizaron 127 sesiones de ET (48 PV y 79 ES) en 20 pacientes (12 ES y 8 PV). La respuesta se obtuvo en el 87,5% de PV y en el 50% de ES. La tasa de complicaciones fue del 7,08%. Conclusiones: A pesar del tamaño de nuestra muestra y la heterogeneidad clínica de nuestra serie, podemos postular que la ET reduce de manera segura los valores de hematocrito en menor tiempo que la flebotomía, especialmente en pacientes con PV y en casos seleccionados de ES en quienes se prevé intolerancia hemodinámica a la flebotomía o en quienes falla este método

Therapeutic erythrocytapheresis (TE) is a more efficient strategy compared to phlebotomy to deplete levels of haematocrit in primary and secondary erythrocytosis. Objective: To analyse response rate and safety profile of TE in polycythemia vera (PV) and secondary erythrocytosis (SE). Patients and method: Retrospective review of all patients with PV or SE treated with TE, due to phlebotomy failure, or comorbidities that prevented changes of blood volumen. Results: 217 TE sessions (48 PV and 79 SE) corresponding to 20 patients (12 ES and 8 PV). Response were achieved in 87.5% of PV patients and in 50% of SE patients. Adverse effects related to TE performance occurred in 7.08%. Conclusion: Despite our small sample size and the heterogeneous nature of the patients included, we can postulate that TE is a secure strategy that can achieve haematocrit depletion in a shorter time than phlebotomy, specifically in PV patients and in selected cases of SE with expected haemodynamic intolerance to phlebotomies or in patients who fail to respond to phlebotomies

Maternal and fetal outcomes in pregnant Japanese women with inflammatory bowel disease: our experience with a series of 23 cases

BACKGROUND/AIMS: Our physicians work to expand the possibilities to treat female patients with inflammatory bowel disease (IBD) who wish to become pregnant. Although many drugs, including 5-aminosalicylate (5-ASA), corticosteroids, immunomodulators, and biologics, are used safely during pregnancy, few reports have described the therapeutic regimen throughout pregnancy and the management of patients who relapse during pregnancy precisely. The aim of this study was to assess the management of patients with IBD during pregnancy. METHODS: We identified 19 patients (five with Crohn's disease and 14 with ulcerative colitis [UC]) who became pregnant with a total of 23 pregnancies between May 2005 and May 2015 by reviewing the medical records of Kyoto University Hospital. The following data were collected: the maternal variables, the IBD treatment type, the disease activity, the pregnancy outcome, and the mode of delivery. RESULTS: Among the 19 patients, 18 had become pregnant after being diagnosed with IBD, while one had developed UC newly after pregnancy. Throughout the gestation, all patients were treated with probiotics, 5-ASA, prednisolone, cytapheresis, or infliximab. The relapse rate during pregnancy was 21.7% (5/23 cases). The five patients who experienced a relapse were able to pursue their pregnancy after intensification of their treatments. There were no adverse fetal or neonatal problems, except in one case that required an emergency Caesarean section because of placental dysfunction and in which a very low-birth-weight infant was born preterm. CONCLUSIONS: Our present data confirmed that even if the disease flares up during pregnancy, good pregnancy outcomes can be achieved with an optimal intensification of the patient's treatment.

Factores predictores de respuesta a las eritroaféresis terapéuticas en pacientes con sobrecarga férrica bioquímica con hemocromatosis hereditaria tipo 1 y sin ella / Predictive factors of response to erytrhocytapheresis in patients with biochemical iron overload with or without hereditary hemochromatosis type 1

Fundamento y objetivo: El aumento progresivo de los depósitos de hierro favorece el desarrollo de diversas entidades, algunas de ellas irreversibles. La piedra angular terapéutica en la sobrecarga férrica ha sido, hasta ahora, la flebotomía. Sin embargo, la eritroaféresis extrae más del doble de hematíes y hierro en cada sesión que una flebotomía convencional, permitiendo alcanzar la depleción férrica en menor tiempo. Los objetivos de este estudio fueron describir las características clínicas y parámetros analíticos de pacientes con sobrecarga férrica tratados mediante eritroaféresis, analizar los resultados globales y por subgrupos, y postular factores predictores de respuesta, así como valorar la seguridad de la técnica. Pacientes y método: Estudio descriptivo, longitudinal y prospectivo de 663 sesiones de eritroaféresis correspondientes a 35 pacientes (entre diciembre de 2002 y octubre de 2011). La respuesta se definió como una cifra de ferritina sérica menor a 50 ng/ml durante dos meses. Para el análisis estadístico se empleó el programa SPSS® versión 17.0 y el nivel mínimo de significación estadística se estableció en un valor de p < 0,05. Resultados: Alcanzaron la respuesta el 77% de los pacientes, con una mediana de 11 (intervalo intercuartílico 1-42) sesiones de eritroaféresis y al cabo de una mediana de 11 (1-108) meses. El 87,5% de los pacientes que no lograron la respuesta redujeron sus valores de ferritina en más del 50%. El descenso en todos los parámetros del metabolismo del hierro fue estadísticamente significativo en el global de pacientes. Fueron factores predictores de respuesta a las eritroaféresis con significación estadística: edad < 60 años, casos de hemocromatosis hereditaria y pacientes con flebotomías previas al inicio de las eritroaféresis. Conclusiones: La eritroaféresis es una técnica efectiva y segura para la depleción férrica en pacientes con sobrecarga de hierro, especialmente en los casos de hemocromatosis hereditaria de alto riesgo que no responden a las flebotomías (AU)

Background and objective: Progressive increase of iron stores leads to the development of varied diseases, some of them irreversible. Until now, phlebotomy has been the cornerstone in the treatment of iron overload. Nevertheless, each erytrhocytapheresis procedure removes more than twice the volume of red cells and iron than phlebotomy, allowing to achieve iron depletion in shorter time. Our aim was to describe clinical features and analytical tests parameters of patients with iron overload, to analyze global and subsets results, to suggest predictive factors of response and to evaluate security of the procedure. Patients and method: Descriptive, longitudinal and prospective study of 663 procedures corresponding to 35 patients (December 2002 to October 2011). Response was defined as a serum ferritine value lower than 50 ng/mL during two months. Statistical analysis was done with SPSS® v 17.0 and the minimum level of statistical significance was defined as p-value < 0,05. Results: Seventy-seven percent of patients reached response with 11 (interquartile range 1-42) erytrhocytapheresis procedures and at 11 (1-108) months. Eighty-seven point five percent of patients who did not achieve response had their ferritine values reduced in more than 50%. The decrease of all iron metabolism parameters was statistically significant. Statistically significant predictive factors of response to erytrhocytapheresis were: patients younger than 60 years-old, hereditary hemochromatosis cases, and patients who had received treatment with phlebotomies prior to erytrhocytapheresis. Conclusions: Erytrhocytapheresis is a secure and effective procedure for iron depletion in patients with iron overload, especially in high risk hereditary hemochromatosis cases that do not respond to phlebotomies (AU)

Alternativas al tratamiento habitual de la hemocromatosis hereditaria / Alternatives to the current treatment of hereditary hemochromatosis

No disponible

Evaluation of methods for collection of peripheral blood stem cells in children / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To evaluate the methods for collection of peripheral blood stem cells (PBSC) in children.</p><p><b>METHODS</b>Peripheral blood stem cells were collected from 20 child patients and 11 donors. The patients treated with chemotherapy, received G-CSF or GM-CSF and the donors received G-CSF for mobilization. When the peripheral blood (PB) leukocyte count reached to 5 X10(9)/L,the hematopoietic stem cells were collected with CS-3000 Plus, COM TEC or COBE Spectra blood cell separators from patients and donors. For children whose weight <20 kg,HCT <24% or TBV <1 100-1 650 ml,blood cell separators were pre-injected with the same type RBCs irradiated by 25 Gy of gamma-ray and with low flow rate (10-30 ml/min). The number of CD34(+) cell was detected by flow cytometry. The relationship of number of CD34(+) cell with mononuclear cell (MNC) and processed blood volume was analyzed.</p><p><b>RESULTS</b>Successful collection of the PBSCs with the CS- 3000 Plus (n=10), the COM TEC (n=3) and the COBE Spectra (n=18) was achieved in all the 31 cases with 1-5 aphereses used. Number of CD34(+) cells was (7.9 ±2.9) X 10(6)/kg and that of MNCs was (7.4 ±3.1) X 10(8)/kg. The total CD34(+) cell count was correlated with MNCs before aphaeresis and processed blood volume.</p><p><b>CONCLUSION</b>For collection of high quality PBSCs, the appropriate methods should be chosen according to the body weight, TBV, mobilization of child patients/donors.</p>

Determination of optimal time to second allogeneic peripheral blood stem cell harvest from healthy donors / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the optimal time for second allogeneic peripheral blood stem cell grafts (PBSC) harvest from healthy donors after in vivo recombinant human granulocyte colony-stimulating factor application (rhG-CSF).</p><p><b>METHODS</b>Thirty-eight healthy donors of second collection (group A) were treated with subcutaneous rhG-CSF \[5 microgxkg(-1)xd(-1)\] for five consecutive days and followed by leukapheresis on day 5 and 6. The control group (group B) was thirty-eight healthy donors who had received a first PBSC collection previously. Group A was reclassified as group C (< or = 9 months) and group D (> 9 months) according to the 75% quantile of interim time between first and second collection. The quantities of lymphocytes of CD3(+), CD3(+)CD4(+), CD3(+)CD8(+), CD14(+), CD34(+) cells and CD3(+)CD4(-)CD8(-) T cells were determined by multi-color flow cytometry.</p><p><b>RESULTS</b>The median number of CD3(+)CD8(+) (25.51 x 10(8)) and CD34(+) cells (0.51 x 10(8)) in group A were significantly lower than that (31.55 x 10(8) and 0.70 x 10(8) respectively) in group B (P < 0.05), and so did the CD3(+)CD8(+) (23.42 x 10(8)) and CD34(+) cells (0.42 x 10(8)) in group C than that in group B (P < 0.05). There was no statistical difference in median numbers of T cell subsets, monocytes, and CD34(+) cells between group B and group D (P > 0.05). The cell ratios of CD4(+)/CD8(+), CD14(+)/CD3(+) and CD3(+)CD4(-)CD8(-) T/CD3(+) in PBSC in group A, group C, and group D were similar to that in group B (P > 0.05). Sperman analysis showed a positive correlation between the total CD34(+) cells in second collection and the interval time from first to second collection (r = 0.357, P = 0.028).</p><p><b>CONCLUSION</b>Nine months after the first collection maybe an optimal time for the second PBSC collection. For those who undergo second PBSC collection within 9 months, more circulation blood should be extracted to ensure enough immunological and hematopoietic compositions.</p>

Cytapheresis in Patients with Severe Ulcerative Colitis after Failure of Intravenous Corticosteroid: A Long-Term Retrospective Cohort Study

BACKGROUND/AIMS: Cytapheresis (CAP) is a novel strategy for ulcerative colitis (UC). However, there is insufficient data on the long-term outcome of UC patients who achieve remission by CAP. This study involved patients with severe UC who refracted to intravenous (iv) corticosteroid. METHODS: Forty-seven UC patients who had received CAP therapy for the first time within 1 year after UC diagnosis were followed for 36 months. One of the inclusion criteria was a clinical activity index (CAI) of > or =7 points at the end of a 2-week iv course of corticosteroid therapy. CAP therapy consisted of ten sessions over 10 weeks. RESULTS: CAP induced clinical remission (CAI or =12, n=25) than for moderately severe UC at entry (7< or =CAI<12, p=15; p<0.02). The cumulative rates of avoiding surgery and relapse were 54.5% and 24.2%, respectively, at 36 months in patients who responded to CAP therapy. This was similar to that of iv cyclosporine reported recently. CONCLUSIONS: This study suggest that CAP is an effective therapy in patients who are refractory to conventional medications including iv corticosteroid. Increased remission rates should be expected in refractory patients with moderately severe UC.

Plasma Exchange in a Patient with Antineutrophil Cytoplasmic Antibody-associated Vasculitis / 대한수혈학회지

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease with a poor prognosis in untreated patients, and combination therapy with steroidsand cyclophosphamide is the current standard treatment. Treatment with immunosuppressive agents results in high remission rates; however, there is a considerable morbidity related to the immunosuppressive state, such as life-threatening infections. To reduce serious immunosuppressant-related adverse effects, potentially promising treatment options, including plasma exchange, cytapheresis, administration of intravenous immunoglobulin and TNF-alpha blocking agents have been developed. We performed plasma exchange with steroid pulse therapy for a 52 year-old female patient with pulmonary hemorrhagewith ANCA-associated crescentic glomerulonephritis. After combination therapy, the pulmonary hemorrhage and renal function recovered and myeloperoxidase (MPO)-ANCA decreased.

Apheresis: the Italian experience / Aférese: a experiência italiana

The Italian experience is a long one, beginning with granulocyte collection in the late Bruni R. months of 1972 and progressively expanding to new application and new techniques, many of which Italian in origin and diffusion. This is true for sequestration, multiple Carlier P. component collection, ascitapheresis, dy-platelet collection, but is also true for the new application of known techniques such as cascade, filtration for disorders such as acute Guillain Barr Syndrome, KT, Cyclosporin induced or secondary hypertriglericeridemia, lepromotous vasculitis leptospirosis, hyperacute kidney rejection, autoimmune pure redcell aplasia and many other disorders treated by plasma exchange for the first time in Italy and in general terms in Italy this intermediate level of complexity techniques have found a wide if not enthusiastic acceptance. Twenty-four years later the general appreciation and interest have not modified their impact onto transfusion medicine and Italy continues to be among the leaders also because of the presence in the country of a couple of industries of international excellence involved in apheresis and/or related fields. The presence of Italy in apheresis was marked by the first international Society (ESFH, European Society for Hemapheresis) set up in 1982, with its first meeting organized in Florence the following year and the one of 2001 that will be held in Italy. The Italian presence in apheresis is also marked by the large Italian participation in the international meetings, frequently as invited speakers and chairmen. Furthermore, many well recognized investigators got their training in Italy and Italy is proud of their achievements.

A experiência italiana em aféreses é antiga e teve início nos últimos meses de 1972 e progressivamente se expandiu com novas aplicações e novas técnicas, muitas delas de origem italiana. Isto é real para sequestração, coleção multipla de componentes, citaféreses e coleção de plaquetas, mas também é verdade que novas aplicações como na sindrome de Guillain-Barré, PTT, hipertrigliceridemia secundária a ciclosporina, vasculite lepromatosa, leptospirose, rejeição renal aguda.