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Paediatric and neonatal transfusions
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
Irradiation should be to a minimum of 25 Gy. If the blood is for top-up only, it may be irradiated up to 14 days from collection and stored for a further 14 days. Blood for exchange transfusion should be irradiated within 5 days of collection and transfused within 24 hours to ensure optimal red cell function and low plasma potassium levels.65 It has been demonstrated that the transfusion of large volumes of irradiated blood 14 days post irradiation contributed to hyperkalaemic cardiac arrest in a child during craniofacial surgery.66 The irradiated units were shown to have a markedly increased potassium concentration of 30 to >40 mmol/l compared with a 3-day-old non-irradiated bag (8.2 mmol/l).
Controlled Clinical Trials — Necessity and Progress
Published in James L. MacPherson, Duke O. Kasprisin, Therapeutic Hemapheresis, 2019
However, it is not yet defined whether plasma infusion alone or plasma exchange is the preferred mode of therapy. Plasma infusion has been advocated by Byrnes who has described five cases of TTP who responded to this modality of therapy alone.8, 9Plasma is presumed to supply the “missing factor” that occurs in TTP which is necessary for the formation of prostacyclin. However, a response to plasma infusion alone does not always occur; Ansell has described a case of a 28-year-old woman with TTP who failed to respond to fresh frozen plasma infusion but subsequently responded to plasma exchange.10 A beneficial response to large volume plasma exchange has now been documented in several series;7, 11, 12, 13, 14 a total of 16 cases recovered completely. In one of these series,7antiplatelet drug therapy was thought to contribute significantly to the response 88% (7 of 8 cases). Whole blood exchange transfusion has also been described to be of benefit, with a clinical remission occurring in 10 of 16 cases described in two reports.15, 16 Plasma exchange or whole blood exchange transfusion is thought to be of benefit because of removal of toxic immune factors such as immune complexes, antiplatelet antibodies or anti-endothelial cell antibodies and this theory is supported by a report of two cases of TTP who responded to plasma exchange with 5% serum albumin and Ringer’s lactate.17 It must be stated that when fresh frozen plasma is used as replacement fluid in TTP, plasma exchange like plasma infusion may serve to replace a “missing plasma factor”.
Haematology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Early diagnosis may be picked up on neonatal screening. Early institution of penicillin prophylaxis is essential and treatment of painful crisis is with plentiful fluids and patient-controlled analgesia. For serious complications, such as chest syndrome and stroke, exchange transfusion is essential at a very early stage. Patients with recurrent severe problems may be considered for bone marrow transplantation.
Recognition of a novel variant of phosphoglycerate kinase 1 deficiency PGK1 Galveston (c.472G > C) in a child with hemolytic anemia, neurologic dysfunction and myopathy
Published in Pediatric Hematology and Oncology, 2023
Edgar Gutierrez, Mathew G. Bayes, Jayati Mallick, Liesel Dell’osso, Kirill A. Lyapichev, Akila Muthukumar
Speculation on a possible relationship between PGK1 deficiency and early-onset parkinsonism has been reported in the previous literature.34,40 Several patients with PGK1 enzyme deficiency have developed early onset parkinsonism.34,40 There are concerning features of the relationship between PGK1 deficiency and parkinsonism that are relevant to the care of our patient. First, the patient with variant (c.1060G > C) developed early onset parkinsonism and was found to have myopathy, anemia, and CNS involvement similar to our patient.31 Moreover, both patients presented in infancy necessitating exchange transfusions and required blood transfusions in infancy. The similarities between these patients must be kept in mind as we continue the long-term neurologic follow-up of our patient. We should also remember that the patient with variant (c.1112T > A) also developed myopathy, hemolytic anemia and neurologic dysfunction but did not have parkinsonism at age 25 years.34 It is interesting to note that both variants that have been associated with parkinsonism are located closer to the C-domain of the PGK1 enzyme. Perhaps there is a relationship to the location of these mutations and the development of parkinsonism in patients with PGK1 deficiency.
Identification and management of fetal anemia due to hemolytic disease
Published in Expert Review of Hematology, 2022
Renske M. van ’t Oever, Carolien Zwiers, Derek de Winter, Masja de Haas, Dick Oepkes, Enrico Lopriore, E.J.(Joanne) Verweij
In utero, excess fetal bilirubin resulting from hemolysis is transported via the placenta into the maternal circulation and is then processed and excreted by the mother. Whether all of the excess fetal bilirubin is transported to the maternal circulation is unknown. Increased bilirubin in utero has been reported and can potentially also lead to neurotoxic damage in the fetus [84]. When the fetus is born, the connection to the maternal system is separated and the neonatal system has to discard the bilirubin by itself. Both the immaturity of the neonatal liver as well as the prolonged hemolysis caused by circulating maternal antibodies contribute to severe hyperbilirubinemia. When left untreated unconjugated bilirubin can cross the blood-brain barrier where it can subside in the basal ganglia, causing acute bilirubin encephalopathy or ‘kernicterus’ [85,86]. Postnatal treatment is focused on preventing bilirubin levels from reaching toxic levels. This is primarily done by intensive phototherapy but sometimes still require exchange transfusions when levels rise above a certain threshold or signs of acute bilirubin encephalopathy occur [80]. During an exchange transfusion, 85% of neonatal blood is replaced with donor blood, removing both excess bilirubin and maternal alloantibodies in the process. Exchange transfusions are however invasive procedures through central lines and are not without risk, requiring sufficient expertise from the treating neonatal team [86].
A Case of Fat Embolism Syndrome with Cerebral Involvement in Sickle Cell Anemia
Published in Hemoglobin, 2021
Rochelle G. Melvin, Zachary Liederman, Sumedha Arya, Lianne Rotin, Christie M. Lee
The management of FES involves supportive care, along with RBC exchange transfusion in patients with sickle cell disease [21,26]. Exchange transfusion works by substituting sickle cell disease RBCs with large numbers of normal erythrocytes [33]. This may halt the ‘sickling cycle’ and prevent further bone marrow necrosis and fat embolization [33]. Simple transfusions (i.e. transfusion of normal erythrocytes without removing sickle cell disease RBCs) provide some benefit but are inferior to exchange transfusions. Multiple simple transfusions can increase blood viscosity and paradoxically enhance the effects of sickling on bone marrow necrosis [27]. A review of FES cases in sickle cell disease demonstrated that mortality was affected by the use of transfusion, as the mortality rate was 23.0, 59.0 and 92.0%, respectively, in patients who received RBC exchange transfusions, simple transfusions and no transfusion, respectively [4,13,16]. It is advised that exchange transfusion be initiated as soon as the diagnosis of FES is suspected, as early intensive exchange transfusion targeting marked reduction in Hb S levels may be life-saving [4,15,27]. For centers that do not have access to exchange transfusion, transfer to a center with apheresis capacity should be facilitated and simple transfusions, up to a Hb of 10.0 g/dL, should be initiated in the interim.