2020
DOI: 10.3324/haematol.2020.253229
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Severe delayed hemolytic transfusion reaction due to anti-Fy3 in a patient with sickle cell disease undergoing red cell exchange prior to hematopoietic progenitor cell collection for gene therapy

Abstract: Severe delayed hemolytic transfusion reaction due to anti-Fy3 in a patient with sickle cell disease undergoing red cell exchange prior to hematopoietic progenitor cell collection for gene therapy.

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Cited by 9 publications
(8 citation statements)
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References 13 publications
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“…It should be noted that patients of African ancestry with the canonical FY GATA mutation can still make alloanti‐Fy3/Fy5 if exposed to Fy b antigen‐positive RBC, as with our patient. 30 , 31 This complex auto‐ and alloimmunization event necessitated multiple work‐ups and send‐out to two different immunohematology reference labs, including our blood supplier's national reference lab.…”
Section: Discussionmentioning
confidence: 99%
“…It should be noted that patients of African ancestry with the canonical FY GATA mutation can still make alloanti‐Fy3/Fy5 if exposed to Fy b antigen‐positive RBC, as with our patient. 30 , 31 This complex auto‐ and alloimmunization event necessitated multiple work‐ups and send‐out to two different immunohematology reference labs, including our blood supplier's national reference lab.…”
Section: Discussionmentioning
confidence: 99%
“…It is well documented that transfusional iron overload is a major cause of toxicity in patients with b-thalassemia, but for reasons that remain incompletely understood, patients with SCD are at relatively lower risk of such iron-related complications. [52][53][54] Nevertheless, transfusions do carry risks, including hemolytic transfusion reactions in SCD, 55,56 underscoring the need for developing optimal transfusion management strategies such as establishment of guidelines for prophylactic matching as well as the number and frequency of units necessary for transfusion for improving the bone marrow niche.…”
Section: Discussionmentioning
confidence: 99%
“…6 It is impor tant for phy si cians car ing for patients with SCD, includ ing those in emer gency depart ments, to be aware that the symp toms of a DHTR may resem ble a vaso occlu sive cri sis. 7 A high degree of sus pi cion for DHTR should be maintained in any patient trans fused in the past month who has symp toms includ ing pain. A com plete blood count (CBC), retic u lo cyte count, chem is tries, LDH, uri nal y sis, Hgb A and S quan ti fi ca tion, type and screen (to eval u ate for any new antibodies in the patient's serum), and direct anti glob u lin test (DAT; to eval u ate antibodies coat ing either the patient's own RBCs or past trans fused RBCs) should be drawn.…”
Section: Pathophysiology Of Delayed Hemo Lytic Trans Fu Sion Reac Tionsmentioning
confidence: 99%
“…There is no "right" answer to the risk/ben e fit ques tion, but this sce nario increas ingly pres ents itself as the num ber of poten tially cura tive ther a pies for SCD rises in num ber. The neces sity of low er ing the Hgb S prior to stem cell trans plan ta tion or periph eral blood stem cell col lec tion (for gene ther apy or for other indi ca tions) is not clear, 7,38 although most pro to cols rec om mend low er ing the Hgb S to decrease trans plantrelated com pli ca tions, to decrease mobi li za tioninduced vasoocclu sion, and/or to improve stem cell mobi li za tion from the mar row. However, patients who have pre vi ously had DHTRs are at increased risk of hav ing future (poten tially lifethreat en ing) DHTRs with RBC expo sure even years later and even when exten sively phe no typ i cally matched RBCs are selected for trans fu sion.…”
Section: Future Trans Fu Sion Con Sid Er Ationsmentioning
confidence: 99%
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