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Hemolytic Anemias: General Considerations
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Delayed hemolytic transfusion reaction is ruled out by the absence of a positive indirect antiglobulin (Coombs) test, and the positive blood cultures imply that this syndrome is due to clostridial hemolysis. The M.D. Anderson Cancer Center has reported an experience of 136 episodes of clostridial bacteremia in cancer patients over a 12-year period. Acute hemolysis was observed infrequently, but was uniformly associated with rapid patient death.
Emerging drugs for the treatment of sickle cell disease: a review of phase II/III trials
Published in Expert Opinion on Emerging Drugs, 2022
Jules M. Ross, Stéphanie Forté, Denis Soulières
Over the last decades, clinical care for SCD patients has been mostly supportive with red cell transfusion and chelation, hydration and analgesia for pain episodes, and immunization [14]. Infectious disease being a major cause of death in infants with SCD, the introduction in 2000 of a vaccine that protects against invasive pneumococcal disease was followed by a 42% reduction of SCD-related death among Black or African-American children younger than 4 years old [15]. Blood transfusion aiming to reduce HbS concentration also remains a mainstay of treatment for many acute and chronic complications of the disease, but it poses significant clinical challenges such as iron overload and alloimmunization. In part because of their erythrocyte phenotype heterogeneity and proinflammatory state, SCD patients are the most allo-immunized of any transfused patient population, facing higher risks of delayed hemolytic transfusion reaction (DHTR) and hyper-hemolysis, and complicating the search for compatible blood [16]. The situation became much more critical with the COVID-19 pandemic, during which more than 80% of the blood supply was lost through cancellations of blood drives, and the blood supply had not returned to its pre-pandemic levels as of January 2021 [17]. In addition, iron overload manifesting with liver and heart disease is not always reversible and requires chelators with hematologic, renal and auditory toxicities [18].
Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management
Published in Expert Review of Hematology, 2022
Elizabeth S. Klings, Martin H. Steinberg
The benefits of transfusion therapy for treatment of ACS have been understood for greater than 30 years [53]. In the absence of controlled clinical trials, which are likely to be impossible to complete, the use of blood transfusion in ACS is guided by expert opinion. Transfusions are at times likely to be lifesaving. Conversely, at times, transfusions are likely to be unnecessary. The minimally affected patient, without hypoxia, with a small segmental infiltrate and little change from ‘steady-state’ blood counts is not likely to need transfusion. This scenario is often observed in pediatric patients and sometimes in adults. Patients with progressive hypoxemia, multilobar disease, progressive anemia, thrombocytopenia and leukocytosis will likely benefit from rapidly instituted exchange transfusion. Between the clinical extremes of ‘benign’ and severe ACS, the choice of whether to use simple or ‘top-up’ transfusion or exchange transfusions is informed by clinical experience, appraisal of a patient’s clinical and laboratory findings and the rapidity with which compatible available blood can be obtained. For mildly hypoxic patients with minor changes in blood counts, simple transfusion will usually suffice; if the patient has HbSC disease and a packed cell volume more than 30, exchange transfusion might be the prudent choice to avoid hyperviscosity. When alloantibodies make transfusion difficult, simple transfusion might be the first choice; history of delayed hemolytic transfusion reaction and the presence of uncharacterized antibodies makes the choice of any transfusion method challenging.
Successful Outcome of Hyperhemolysis in Sickle Cell Disease following Multiple Lines of Treatment: The Role of Complement Inhibition
Published in Hemoglobin, 2018
Efthymia Vlachaki, Eleni Gavriilaki, Katerina Kafantari, Despoina Adamidou, Dimitris Tsitsikas, Eleni Chasapopoulou, Achilles Anagnostopoulos, Apostolos Tsapas
Hyperhemolysis is a well-recognized, yet potentially life-threatening complication of transfusions predominantly observed in sickle cell disease. It is described as acute (occurring less than 5 days after transfusion) or delayed (5 to 20 days after transfusion). Although delayed hemolytic transfusion reaction (DHTR) with hyperhemolysis may be catastrophic within a few hours, its diagnosis remains challenging as it presents with symptoms of vaso-occlussive crisis (VOC) [1]. Furthermore, alloimmunization is considered the main cause of DHTR, though antibodies cannot be detected in about one-third of DHTR cases [2,3]. In this setting of difficult diagnosis and unclear pathophysiology, there is still no optimal treatment for these patients. Complement inhibition with the anti-C5 monoclonal antibody, eculizumab, has recently been reported as a successful salvage treatment in five patients [4–6]. However, optimal timing, patient selection based on complement activation assessment and eculizumab dosing remain unanswered questions in the field. We report evidence of complement activation and successful complement inhibition with one dose of eculizumab following multiple lines of treatment in an adult sickle cell disease patient presenting DHTR with hyperhemolysis.