American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support.

BACKGROUND: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. OBJECTIVE: Our objective was to develop evidence-based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion-related complications. METHODS: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. RESULTS: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. CONCLUSIONS: The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.

Transfusion-associated circulatory overload risk mitigation: survey on hospital policies for compliance with AABB Standard 5.9.17.

BACKGROUND: AABB Standards for Blood Banks and Transfusion Services require accredited institutions to have a policy for handling requests for blood components on patients clinically identified as being at high risk for transfusion-associated circulatory overload (TACO; Standard 5.19.7, 31st edition). This survey elucidated how AABB accredited hospital transfusion services/blood banks around the world are complying with this Standard. METHODS: A link to a Web-based survey in English was e-mailed under the auspice of the AABB to each AABB accredited hospital transfusion service/blood bank (n = 851) asking for details on how their institution is complying with this Standard and for general information on any TACO risk mitigation strategies in place. RESULTS: Of the 290 responses received (34% response rate), 282 met the criteria for analysis. There were 174 of 282 (62%) respondents who indicated that their institution has a formal policy for complying with the Standard, and 108 of 282 (38%) who indicated that their institution does not have a formal policy. A diverse range of policies and practices were in place at the institutions with and without a formal policy ranging from writing advice/recommendations in the charts of patients at increased risk of TACO, promulgating policies from the transfusion service/blood bank or institution itself that would reduce the risk, or using decision support tools to provide education about reducing the risk of TACO. CONCLUSIONS: Many but not all AABB accredited institutions have policies to comply with the TACO risk mitigation Standard. However, the vast majority conduct activities that could mitigate risk for TACO.

Reacción hemolítica transfusional tardía en un paciente con anemia de células falciformes: reporte de un caso / Delayed hemolytic reaction to transfusion in sickle cell anemia: report of one case

Sickle cell anemia was a rare disease in Chile, especially in adults, however the recent immigration wave from Haiti is changing this scenario. We report a 29 year old black female from Haiti with a non-disclosed history of sickle cell anemia. She was transfused with two units of red blood cells, found unconscious and with jaundice five days later and admitted to the hospital. On admission she had a hemoglobin of 3.3 g/dL, a total bilirubin of 5.08 mg/dL, a LDH of 1,306 Ui/L. She was transfused again, worsening her condition. An alloimmunization and delayed hemolytic reaction was suspected. A direct Coombs test was positive. She was treated with steroids and her serum hemoglobin rose progressively.

[Delayed hemolytic reaction to transfusion in sickle cell anemia. Report of one case]. / Reacción hemolítica transfusional tardía en un paciente con anemia de células falciformes: reporte de un caso.

Sickle cell anemia was a rare disease in Chile, especially in adults, however the recent immigration wave from Haiti is changing this scenario. We report a 29 year old black female from Haiti with a non-disclosed history of sickle cell anemia. She was transfused with two units of red blood cells, found unconscious and with jaundice five days later and admitted to the hospital. On admission she had a hemoglobin of 3.3 g/dL, a total bilirubin of 5.08 mg/dL, a LDH of 1,306 Ui/L. She was transfused again, worsening her condition. An alloimmunization and delayed hemolytic reaction was suspected. A direct Coombs test was positive. She was treated with steroids and her serum hemoglobin rose progressively.