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Erythrocytapheresis

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https://www.readbyqxmd.com/read/29301288/use-of-pleuroperitoneal-shunt-in-chylothorax-related-to-central-line-associated-thrombosis-in-sickle-cell-disease
#1
Elizabeth Spiwak, Chad Wiesenauer, Arun Panigrahi, Ashok Raj
Central vein thrombosis as a cause of chylothorax is uncommon, and in a few cases in the literature was related to thrombotic complications of central venous access devices (CVAD). Superior vena cava (SVC) occlusion-induced chylothorax has been described in adult sickle cell disease (SCD) in a setting of chronic indwelling CVAD. There are limited reports on chylothorax induced by central venous thrombosis secondary to chronic CVAD in children with SCD. We describe an 8-year-old male patient, with a history of SCD, maintained on long term erythrocytapheresis for primary prevention of stroke, and whose clinical course was complicated by chylothorax which was successfully treated with a pleuroperitoneal shunt...
January 2, 2018: Children
https://www.readbyqxmd.com/read/29195602/reduction-of-body-iron-in-hfe-related-haemochromatosis-and-moderate-iron-overload-mi-iron-a-multicentre-participant-blinded-randomised-controlled-trial
#2
Sim Y Ong, Lyle C Gurrin, Lara Dolling, Jeanette Dixon, Amanda J Nicoll, Michelle Wolthuizen, Erica M Wood, Gregory J Anderson, Grant A Ramm, Katrina J Allen, John K Olynyk, Darrell Crawford, Louise E Ramm, Paul Gow, Simon Durrant, Lawrie W Powell, Martin B Delatycki
BACKGROUND: The iron overload disorder hereditary haemochromatosis is most commonly caused by HFE p.Cys282Tyr homozygosity. In the absence of results from any randomised trials, current evidence is insufficient to determine whether individuals with hereditary haemochromatosis and moderately elevated serum ferritin, should undergo iron reduction treatment. This trial aimed to establish whether serum ferritin normalisation in this population improved symptoms and surrogate biomarkers. METHODS: This study was a multicentre, participant-blinded, randomised controlled trial done at three centres in Australia...
December 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29138743/renal-adenocarcinoma-associated-erythrocytosis-in-a-cat-clinicopathological-features-and-immunohistochemical-results
#3
Roberta Troia, Chiara Agnoli, Federico Fracassi, Giuliano Bettini, Alessandra Sfacteria, Luciano Pisoni, Francesco Dondi
This report documents a case of secondary inappropriate erythrocytosis in a cat with renal cell adenocarcinoma, its stabilization through manual erythrocytapheresis, and the EPO-immunostaining on the affected kidney. An 11-year-old cat was presented with lethargy, weight loss and polyuria/polydipsia. An abdominal mass was detected upon physical examination. Clinicopathological work-up revealed marked erythrocytosis (HCT value 64.8%), renal azotemia and decreased urine specific gravity (USG). An abdominal ultrasound was performed, localizing the mass in the right kidney...
2017: Open Veterinary Journal
https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#4
Françoise Bernaudin, Corinne Pondarré, Claire Galambrun, Isabelle Thuret
Allogeneic hematopoietic stem cell transplantation (HSCT) can cure single gene disorders such as thalassemia and sickle cell anemia (SCA). These non-malignant diseases have in common severe hemolytic anemia and high proliferative bone marrow, requiring frequent transfusions. The risk of rejection is high and graft-vs-host disease is not desirable. Important progress has been made in the management of these diseases, including leukocyte depletion of blood products, and chelation therapy, for both diseases, and erythrocytapheresis and hydroxycarbamide for SCA...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28695837/a-case-of-cerebral-malaria-managed-by-erythrocyte-exchange
#5
Gürsel Ersan, Işıl Köse, Fatma Liv, Bengü Gireniz Tatar, Şükran Köse
Plasmodium falciparum may cause serious and sometimes even fatal cerebral malaria. Cerebral malaria is a disease characterized by alterations in consciousness and epileptic attacks; therefore, it requires a rapid evaluation and quick intervention. It may progress to a fatal outcome despite administering targeted therapeutic interventions. There are studies indicating that erythrocyte exchange (erythrocytapheresis) can be a treatment modality for managing severe malaria patients. In this case report, the successful management of a cerebral malaria patient by the combination of antimalarial drug therapy and erythrocyte exchange is presented...
June 2017: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/28544309/increased-complications-of-chronic-erythrocytapheresis-compared-with-manual-exchange-transfusions-in-children-and-adolescents-with-sickle-cell-disease
#6
Deborah Woods, Robert J Hayashi, Michael M Binkley, Gianna W Sparks, Monica L Hulbert
BACKGROUND: Children and adolescents with sickle cell disease (SCD) are at high risk of strokes and are frequently treated with red blood cell (RBC) transfusions. The goal is to suppress hemoglobin (Hb) S while minimizing transfusion-induced iron overload. RBCs may be given via simple transfusion, manual exchange transfusion (MET), or erythrocytapheresis (aRBCX). Chronic transfusion practices vary among institutions. METHODS: This single-institution, retrospective cohort study compares Hb S control and therapy complication rates between MET and aRBCX in a cohort of children and adolescents with SCD and stroke during a 5-year period from 2008 through 2012...
November 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28470719/from-total-blood-exchange-to-erythrocytapheresis-and-back-to-treat-complications-of-sickle-cell-disease
#7
Samir K Ballas
Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient...
September 2017: Transfusion
https://www.readbyqxmd.com/read/28273330/interventions-for-hereditary-haemochromatosis-an-attempted-network-meta-analysis
#8
REVIEW
Elena Buzzetti, Maria Kalafateli, Douglas Thorburn, Brian R Davidson, Emmanuel Tsochatzis, Kurinchi Selvan Gurusamy
BACKGROUND: Hereditary haemochromatosis is a genetic disorder related to proteins involved in iron transport, resulting in iron load and deposition of iron in various tissues of the body. This iron overload leads to complications including liver cirrhosis (and related complications such as liver failure and hepatocellular carcinoma), cardiac failure, cardiac arrhythmias, impotence, diabetes, arthritis, and skin pigmentation. Phlebotomy (venesection or 'blood letting') is the currently recommended treatment for hereditary haemochromatosis...
March 8, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27813144/arterio-venous-fistula-for-automated-red-blood-cells-exchange-in-patients-with-sickle-cell-disease-complications-and-outcomes
#9
Marianne Delville, Sandra Manceau, Nassim Ait Abdallah, Jan Stolba, Sameh Awad, Thibaud Damy, Barnabas Gellen, Laurent Sabbah, Karima Debbache, Vincent Audard, Jean-Louis Beaumont, Cécile Arnaud, Christelle Chantalat-Auger, Françoise Driss, François Lefrère, Marina Cavazzana, Gilbert Franco, Frederic Galacteros, Jean-Antoine Ribeil, Justine Gellen-Dautremer
Erythrocytapheresis (ER) can improve outcome in patients with sickle cell disease (SCD). A good vascular access is required but frequently it can be difficult to obtain for sickle cell patients. Arterio-venous fistulas (AVFs) have been suggested for ER in SCD supported by limited evidence. We report the largest cohort of ER performed with AVFs from three French SCD reference centers. Data of SCD patients undergoing ER with AVFs in the French SCD reference center were retrospectively collected. The inclusion criteria were: SS or Sβ-Thalassemia and AVF surgery for ER...
February 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27789107/successful-treatment-of-iron-overload-cardiomyopathy-in-hereditary-hemochromatosis-with-deferoxamine-and-deferiprone
#10
Lydie Tauchenová, Barbora Křížová, Miloš Kubánek, Soňa Fraňková, Vojtěch Melenovský, Jaroslav Tintěra, Dana Kautznerová, Jana Malušková, Milan Jirsa, Josef Kautzner
There is scarce evidence regarding the use of iron chelators in patients with hereditary hemochromatosis who are intolerant of phlebotomy or erythrocytapheresis. A 52-year-old man with genetically confirmed HFE hemochromatosis presented with liver disease and heart failure with severe left ventricular systolic dysfunction. Because of anemia after initial treatment, we added intravenous deferoxamine followed by oral deferiprone to less frequent erythrocytapheresis, which normalized systolic function within 1 year...
December 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27759170/erythrocytapheresis-for-chronic-transfusion-therapy-in-sickle-cell-disease-survey-of-current-practices-and-review-of-the-literature
#11
REVIEW
Shannon Kelly, Keith Quirolo, Anne Marsh, Lynne Neumayr, Alicia Garcia, Brian Custer
BACKGROUND: Chronic red blood cell (RBC) transfusion therapy (CTT) is an integral component of the management of severe sickle cell disease (SCD) and can prevent complications, such as stroke. RBC units can be administered via simple transfusion or exchange transfusion, and erythrocytapheresis (automated RBC exchange transfusion [aRBX]), is increasingly used for CTT. Comparisons of simple and aRBX transfusions are limited, and the current scope of aRBX use is not known. STUDY DESIGN AND METHODS: We administered a survey to define current transfusion practices for CTT and performed a review of the erythrocytapheresis literature...
November 2016: Transfusion
https://www.readbyqxmd.com/read/27723100/how-we-manage-patients-with-hereditary-haemochromatosis
#12
REVIEW
Eva Rombout-Sestrienkova, Marian G J van Kraaij, Ger H Koek
A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of erythrocytapheresis. In general, therapy is based on the removal of excess body iron, for which ferritin levels are used to monitor the effectiveness of treatment. For many decades phlebotomy has been widely accepted as the standard treatment...
December 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27271482/efficacy-and-safety-of-erythrocytapheresis-and-low-dose-erythropoietin-for-treatment-of-hemochromatosis
#13
Dorothea Brückl, Sundrela Kamhieh-Milz, Julian Kamhieh-Milz, Abdulgabar Salama
BACKGROUND: The aim of this study was to determine retrospectively the efficacy of combined therapy using erythropoietin (EPO) and erythrocytapheresis (EA) in patients with hereditary hemochromatosis (HH) who did not tolerate phlebotomy. PATIENTS AND METHODS: Twenty patients (age range, 43-74 years) with genetically confirmed HH had received low-dose EPO (4,000 IU) in accordance to the patient's hemoglobin levels between each EA session. Laboratory parameters including hemoglobin, ferritin, transferrin, and iron were measured at regular intervals...
June 8, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27100139/transfusional-iron-overload-in-a-cohort-of-children-with-sickle-cell-disease-impact-of-magnetic-resonance-imaging-transfusion-method-and-chelation
#14
Helen M Stanley, David F Friedman, Jennifer Webb, Janet L Kwiatkowski
BACKGROUND: Transfusions prevent a number of complications of sickle cell disease (SCD), but cause inevitable iron loading. With magnetic resonance imaging (MRI), liver iron can be monitored noninvasively. Erythrocytapheresis can limit iron loading and oral chelation provides a more tolerable alternative to subcutaneous administration. The impact of these factors on control of iron burden in SCD has not been well studied. PROCEDURE: Iron monitoring practices, chelation use, and transfusion methods were assessed in our cohort of pediatric patients with SCD receiving chronic transfusion...
August 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27021622/comparison-of-automated-erythrocytapheresis-versus-manual-exchange-transfusion-to-treat-cerebral-macrovasculopathy-in-sickle-cell-anemia
#15
COMPARATIVE STUDY
Bérengère Koehl, Julie Sommet, Laurent Holvoet, Hendy Abdoul, Priscilla Boizeau, Ghislaine Ithier, Florence Missud, Nathalie Couque, Suzanne Verlhac, Pauline Voultoury, Fatiha Sellami, André Baruchel, Malika Benkerrou
BACKGROUND: Chronic exchange transfusion is effective for primary and secondary prevention of stroke in children with sickle cell anemia (SCA). Erythrocytapheresis is recognized to be the most efficient approach; however, it is not widely implemented and is not suitable for all patients. The aim of our study was to compare automated exchange transfusion (AET) with our manual method of exchange transfusion and, in particular, to evaluate the efficacy, safety, and cost of our manual method...
May 2016: Transfusion
https://www.readbyqxmd.com/read/26997031/effectiveness-of-red-blood-cell-exchange-partial-manual-exchange-and-simple-transfusion-concurrently-with-iron-chelation-therapy-in-reducing-iron-overload-in-chronically-transfused-sickle-cell-anemia-patients
#16
Ross M Fasano, Traci Leong, Megha Kaushal, Eyal Sagiv, Naomi L C Luban, Emily Riehm Meier
BACKGROUND: Chronic transfusion therapy (CTT) is indicated for stroke prevention in children with sickle cell anemia (SCA) and is complicated by iron overload and alloimmunization. CTT is performed by simple transfusion (ST), partial manual exchange (PME), or erythrocytapheresis (RCE). Although small case series have demonstrated RCE in combination with iron chelation therapy stabilizes and/or decreases ferritin, there are no reports comparing the effect of ST, PME, and RCE on liver iron concentration (LIC)...
July 2016: Transfusion
https://www.readbyqxmd.com/read/26992127/the-modified-iron-avidity-index-a-promising-phenotypic-predictor-in-hfe-related-haemochromatosis
#17
Pauline L M Verhaegh, Wenke Moris, Ger H Koek, Cees Th B M van Deursen
OBJECTIVE: Phenotypes of the HFE-related haemochromatosis vary considerably, making it hard to predict the course of iron accumulation. The aim of this retrospective study was to determine if the Iron Avidity Index (IAI) is a good phenotypic predictor of the number of phlebotomies needed per year during maintenance treatment (NPDMT) in patients with homozygous p.C282Y hereditary haemochromatosis (HH). METHODS: Patients with HH homozygous for p.C282Y, on maintenance treatment for at least 1 year were included...
October 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/26878994/course-of-iron-parameters-in-hfe-hemochromatosis-patients-during-initial-treatment-with-erythrocytapheresis-compared-to-phlebotomy
#18
COMPARATIVE STUDY
Eva Rombout-Sestrienkova, Ger H Koek, Rabin Neslo, Marian van Kraaij, Paul P Menheere, Ad Masclee, Dorine W Swinkels
Current treatment for newly diagnosed patients with hereditary hemochromatosis (HH) and iron overload consist of weekly phlebotomy or less frequent and more personalized erythrocytapheresis. Previous observations during phlebotomy suggest an increase in intestinal iron uptake caused by lowering of hepcidin as a result of intensive bloodletting. It is not known whether such an effect is present or even more pronounced using erythrocytapheresis since a larger amount of iron is extracted per procedure. In this study we aimed to assess the effect of erythrocytapheresis on the course of iron parameters, with special focus on serum hepcidin...
December 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/26650249/long-term-erythrocytapheresis-is-associated-with-reduced-liver-iron-concentration-in-sickle-cell-disease
#19
Scott N Myers, Ryan Eid, John Myers, Salvatore Bertolone, Arun Panigrahi, Jennifer Mullinax, Ashok B Raj
BACKGROUND: Erythrocytapheresis procedures are increasingly used in sickle cell disease. Serum ferritin and noninvasive magnetic resonance imaging measurements of liver iron concentration (LIC) are frequently used to monitor iron overload secondary to hypertransfusion. There is a paucity of data describing the impact of long-term erythrocytapheresis (LTE) on LIC. MATERIALS AND METHODS: We measured magnetic resonance imaging liver and cardiac iron on LTE subjects and stratified them into 2 groups: higher LIC (>3 mg/g) and lower LIC (<3 mg/g)...
January 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/26358375/erythrocytapheresis-versus-phlebotomy-in-the-maintenance-treatment-of-hfe-hemochromatosis-patients-results-from-a-randomized-crossover-trial
#20
RANDOMIZED CONTROLLED TRIAL
Eva Rombout-Sestrienkova, Bjorn Winkens, Brigitte A B Essers, Fred H M Nieman, Paulus A H Noord, Mirian C H Janssen, Cees Th B M van Deursen, Annelies Boonen, Ellen P J M Reuser-Kaasenbrood, Judith Heeremans, Marian van Kraaij, Ad Masclee, Ger H Koek
BACKGROUND: Phlebotomy is standard maintenance treatment of patients with hereditary hemochromatosis (HH). Erythrocytapheresis, which selectively removes red blood cells, provides a new, potentially more effective treatment option. Our aim was to evaluate the effectiveness of erythrocytapheresis over phlebotomy for maintenance therapy in patients with HH. STUDY DESIGN AND METHODS: We conducted a two-treatment-arms, randomized, crossover clinical trial, involving 46 patients, treated for 1 year with either erythrocytapheresis or phlebotomy to keep the ferritin level at not more than 50 µg/L...
January 2016: Transfusion
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