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Sickle cell disease
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Marc R. Parrish, John C. Morrison
Exchange transfusion, also known as erythrocytapheresis, allows the Hb S-containing cells and irreversibly sickled cells to be removed by extracorporeal, differential centrifugation. It affords the simultaneous return by venous access in the other arm of the patient’s own plasma, leukocytes, platelets, and clotting factors along with donor, leukocyte-poor washed Hb A-containing red cells. This type of transfusion occurs via a machine that allows for automated continuous erythrocytapheresis. In addition, this method maintains isovolemia at all times and allows the provider to accurately monitor the patient’s hematologic indices such as hemoglobin levels and hematocrit. It also has the advantage of administration on an outpatient basis. If such a device is not available, the manual “push–pull” mechanism can be considered (Table 4) (45).
Therapeutic apheresis
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
In 1914, Abel, Rowntree and Turner1 coined the term plasmaphaeresis (from the Greek word aphairesis – a withdrawal). Their early experiments were for the relief of symptoms following bilateral nephrectomy in dogs. Although these experiments were associated with deaths (due to apparent overbleeding and hemorrhage), the improvement in the clinical condition of the animals successfully treated was ‘marked’. The term apheresis has since been generalized to refer to the separation of blood into its components, removing one component, and returning the remainder. Thus, leukapheresis means the removal of leukocytes and erythrocytapheresis means the removal of erythrocytes. Alternative terminologies such as plasma exchange and red cell exchange are frequently used interchangeably for plasmapheresis and erythrocytapheresis, respectively. Some authors have suggested that the term ‘plasma exchange’ or therapeutic plasma exchange be reserved for low-volume procedures involving no more then 500–600 ml of plasma and plasmapheresis for large-volume procedures; however, these terms are frequently used interchangeably. Hemapheresis is also used as a broad term encompassing all apheresis procedures.
Management of sickle cell disease: current practices and challenges in a northeastern region of the Democratic Republic of the Congo
Published in Hematology, 2021
Paul Kambale-Kombi, Roland Marini Djang’eing’a, Jean-Pierre Alworong’a Opara, Jean-Marc Minon, François Boemer, Vincent Bours, Serge Tonen-Wolyec, Charles Kayembe Tshilumba, Salomon Batina-Agasa
None of the patients were in a chronic transfusion program. A number of factors can account for this result. The lack of transcranial Doppler equipment to identify children with SCD at risk of stroke, the lack of erythrocytapheresis equipment and the absence of quantitative Hemoglobin S(HbS) and Hemoglobin F(HbF) assays in our area do not make it possible to consider a chronic transfusion program. In addition, the persistence of family and remunerated blood donors is a potential barrier to chronic transfusion in our setting, since a high risk of transfusion-transmitted infections has been reported in this category of donors [24,25]. Moreover, the guidelines recommend to irradiate donations from first or second-degree relatives, even if the patient is immunocompetent, to prevent transfusion-associated graft-vs-host disease (TA-GVHD) [26]. However, data regarding TA-GVHD are not available in DRC.
Veno-Venous Extracorporeal Membrane Oxygenation in Adult Patients with Sickle Cell Disease and Acute Chest Syndrome: a Single-Center Experience
Published in Hemoglobin, 2020
Ferras Alashkar, Frank Herbstreit, Alexander Carpinteiro, Julia Baum, Asterios Tzalavras, Carmen Aramayo-Singelmann, Colin Vance, Veronika Lenz, Erich Gulbins, Dirk Reinhardt, Dietrich W. Beelen, Ulrich Dührsen, Alexander Röth, Michael Koldehoff, Tobias Liebregts
Simple transfusions should be restricted to symptomatic patients (e.g. patients presenting with a PaO2 <9.0 kPa on room air or if the Hb concentration is >1.0 g/dL below baseline) to improve oxygenation. In patients with a rapid clinical deterioration characterized by a further Hb decline despite prior simple transfusions or in patients with a Hb >9.0 g/dL and evidence of increased respiratory distress, progressive pulmonary infiltrates, or an oxygen saturation of <90.0% despite supplemental oxygen therapy, immediate initiation of exchange transfusions (isovolemic manual exchange or automated erythrocytapheresis) is implicated and to be maintained until recovery [1,5]. Prior to transfusion initiation, an advanced phenotyping matching for ABO and RBC antigens (D, Cc, Ee, Kell, Kidd, and Duffy) is required as patients are at risk for allo- and autoimmunization and/or delayed hemolytic transfusion reactions (DHTR) [1,13–15].