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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
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...
May 19, 2017: Pediatric Blood & Cancer
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...
May 3, 2017: Transfusion
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
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
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
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
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
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
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
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
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
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
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
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
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
Bruno Deltreggia Benites, Thais Celi Lopes Benevides, Isabella Salvetti Valente, Jose Francisco Marques, Simone Cristina Olenscki Gilli, Sara Teresinha Olalla Saad
BACKGROUND: Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease. MATERIALS AND METHODS: Patients were divided into two groups according to the type of transfusion support received: 10 women received prophylactic erythrocytapheresis or manual exchange transfusion at 28 weeks of gestation, and 14 received transfusions only on demand, due to acute complications, or received no transfusions at all...
January 2016: Transfusion
Sim Yee Ong, Lara Dolling, Jeannette L Dixon, Amanda J Nicoll, Lyle C Gurrin, Michelle Wolthuizen, Erica M Wood, Greg J Anderson, Grant A Ramm, Katrina J Allen, John K Olynyk, Darrell Crawford, Jennifer Kava, Louise E Ramm, Paul Gow, Simon Durrant, Lawrie W Powell, Martin B Delatycki
INTRODUCTION: HFE p.C282Y homozygosity is the most common cause of hereditary haemochromatosis. There is currently insufficient evidence to assess whether non-specific symptoms or hepatic injury in homozygotes with moderately elevated iron defined as a serum ferritin (SF) of 300-1000 µg/L are related to iron overload. As such the evidence for intervention in this group is lacking. We present here methods for a study that aims to evaluate whether non-specific symptoms and hepatic fibrosis markers improve with short-term normalisation of SF in p...
August 12, 2015: BMJ Open
Sindy Karina Ibeth Domínguez-Morales, Luis Carlos Moreno-López, Juan Manuel Gallardo, José Ramón Paniagua
BACKGROUND: Although automated cell separators (apheresis) have undergone a lot of technical refinements, the effect of the procedure on hematological indices of donors is rarely taken into account. The purpose of this study is to identify potential hematologic changes in donors undergoing erythrocytapheresis. METHODS: 30 apparently healthy adult donors were evaluated. Erythrocytapheresis procedure was performed using automated equipment. Hematologic measurements (hemoglobin, hematocrit, white blood cells counts and platelets) were analyzed before and after erythrocytapheresis in all donors...
July 2015: Revista Médica del Instituto Mexicano del Seguro Social
Burcu Camcıoğlu, Meral Boşnak-Güçlü, Müşerrefe Nur Karadallı, Şahika Zeynep Akı, Gülsan Türköz-Sucak
Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement...
2015: Case Reports in Hematology
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