Delayed Hemolytic Transfusion Reaction Presenting as Sickle-Cell Crisis

Diamond, W. John; Brown, F. L.; Bitterman, Peter B.; Klein, Harvey G.; Davey, Richard J.; Winslow, Robert M.

doi:10.7326/0003-4819-93-2-231

Cited by 104 publications

(49 citation statements)

References 8 publications

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“…Hemoglobin S (Hb s ), containing the mutated β-globin, causes changes in rbc shape, stiffness, and adhesiveness, thereby altering rheological properties and inducing hemolysis and vaso-occlusion (VOC) (1)(2)(3). Although rbc transfusions correct anemia, improve rheological properties, and increase oxygen-carrying capacity in SCD patients, they can produce potentially dangerous immunological responses as a result of repeated exposure to allogeneic blood group antigens (4)(5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

CXCL1 and its receptor, CXCR2, mediate murine sickle cell vaso-occlusion during hemolytic transfusion reactions

et al. 2011

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Hemolytic transfusion reactions (HTR) can produce acute vaso‐occlusive crisis (VOC), a major cause of morbidity and mortality in sickle cell disease (SCD) patients; however, the mechanisms remain unknown. We established a model of alloimmune IgG‐mediated HTR using a well‐characterized humanized murine model of SCD. In this model, HTR induced acute VOC, resulting in shortened survival of the SCD mice (by 3 h, P<0.05, log‐rank test). Acute VOC was associated with elevated circulating inflammatory chemokine levels, including the neutrophil chemoattractant CXCL1, which was strikingly elevated (139 ± 35 vs 43 ± 10 pg/ml, P<0.05). Recombinant CXCL1 administration corresponding to the HTR‐elicited endogenous CXCL1 levels induced acute VOC in non‐transfused SCD mice, characterized by leukocyte recruitment in venules (2571 ± 193 vs 1464 ± 194 adherent leukocytes per mm2, P<0.01), marked interactions with circulating red blood cells, reduced venular flow rates (430 ± 34 vs 310 ± 36 nL/s, P<0.05), and shortened survival (by 4 h, P<0.001, log‐rank test). In contrast, blocking its receptor, CXCR2, prevented HTR‐elicited acute VOC and prolonged the survival of SCD mice (by 6 h, P<0.01, log‐rank test). Thus, CXCL1 is a key inflammatory mediator of acute VOC in SCD mice and targeted inhibition of CXCL1 and/or CXCR2 may provide new approaches for treating acute VOC in SCD patients.

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Section: Introductionmentioning

confidence: 99%

CXCL1 and its receptor, CXCR2, mediate murine sickle cell vaso-occlusion during hemolytic transfusion reactions

et al. 2011

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“…The main RBC alloantibodies im plicated in DHTR in patients with SCD are anti-C, anti-E, anti-Jkb and anti-K [4]. In the majority of previously de scribed cases of DHTR in SCD the antibodies implicated in the reactions were clearly demonstrable in the serum of the recipient several days after the transfusion [4][5][6], The pa tient we have described did not develop any previously un detected antibodies, and only transiently developed a weak ly positive DAT (IgG alone) following the administration of IVIg: the eluate was nonreactive. The positive DAT seems likely to have been caused by IVIg [9,10].…”

Section: G/kg/daymentioning

confidence: 99%

“…and the presence in serum of previously undetected RBC alloantibodies, together with clinical evidence of haemoly sis [1,2], Although clinical evidence of haemolysis is com mon in DHTR, this is not usually severe and death from DHTR is rare [1,2]. In SCD the clinical picture of DHTR is modified [5,6]: clinically significant alloimmunisation to RBC antigens occurs in 20-25% of transfused patients with SCD [4,8] and the incidence of DHTR appears higher than that in other patients, 11% in one series [4]. In many SCD patients, as in the case described, multiple red cell alloanti bodies may be present, making provision of compatible blood a major problem.…”

Section: G/kg/daymentioning

confidence: 99%

“…DHTRs are commoner in patients with sickle cell disease (SCD) [4] and the clinical manifestations can be different from those described in oth er patients, and patients can present with symptoms that mimic those of a vaso-occlusive sickle cell crisis or with life-threatening severe haemolytic anaemia [5,6], A form of severe hyperhaemolysis associated with trans fusion has recently been described in children with SCD <£' 1995 S Karger AG, Basel 0042-9007/95'0694-0355 $8 00'0 Case Report IVIg …”

Section: Introductionmentioning

confidence: 99%

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Post-Transfusion Hyperhaemolysis in a Patient with Sickle Cell Disease: Use of Steroids and Intravenous Immunoglobulin to Prevent Further Red Cell Destruction

Dudley¹,

Kaye²,

Cullis³

et al. 1995

Vox Sanguinis

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Delayed haemolytic transfusion reactions (DHTRs) are seen more frequently in patients with sickle cell disease (SCD) than in other groups of patients, and are characterised by a positive direct antiglobulin test and the appearance of previously undetected red blood cell (RBC) alloantibodies in the patient’s serum. Recently a syndrome of post-transfusion hyperhaemolysis has been described in children with SCD, characterised by destruction of both autologous and transfused RBCs with negative serological findings: continuation of RBC transfusion exacerbated haemolysis further. We describe a case of life-threatening posttransfusion hyperhaemolysis in an adult patient with SCD in whom severe anaemia necessitated further RBC transfusion, which was successfully performed in conjunction with intravenous immunoglobulin. This approach may be useful in the management of post-transfusion hyperhaemolysis in SCD as well as in the management of severe DHTRs.

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“…In addition, alloimmunized SCD patients have an increased risk of experiencing a hemolytic transfusion reaction (HTR) that occurs when preexisting alloantibodies become undetectable and the patient receives crossmatch compatible blood that is antigen-positive. These HTRs in patients with SCD may be mistaken for sickle cell crisis and therefore go unrecognized [3]. In addition, there is also 0 1993 Wiley-Liss, Inc.…”

mentioning

confidence: 99%

A simple, practical model for reducing alloimmunization in patients with sickle cell disease

et al. 1993

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Patients with sickle cell disease (SCD) form immune alloantibodies more frequently than other transfused populations because red cells (RBCs) from white donors (with a higher incidence of certain Rh, Duffy, Kell, and Kidd blood group antigens) are transfused to black patients often lacking these antigens. We propose a model to reduce alloimmunization in patients with SCD by providing them with blood from only black random donors. Rationale is shown by examining calculations based on the phenotype E-, C-, Fy(a-), K-, and Jk(b-). There is a 7% probability that this phenotype belongs to a white donor, while there is a 93% probability that this phenotype belongs to a black donor. The probability of selecting blood from a black donor identical with the above phenotype for black recipients from an all black population and from a typical urban blood inventory population (90% white, 10% black) is 1/4 and 1/33, respectively. Therefore, an 8-fold greater chance of selecting antigen non-identical blood occurs if blood is obtained from a typical urban donor population as compared to a black population. Based on these calculations, alloimmunization can be reduced prospectively in patients with SCD by meeting their transfusion requirements with blood selected from random black blood donors.

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Delayed Hemolytic Transfusion Reaction Presenting as Sickle-Cell Crisis

Cited by 104 publications

References 8 publications

CXCL1 and its receptor, CXCR2, mediate murine sickle cell vaso-occlusion during hemolytic transfusion reactions

CXCL1 and its receptor, CXCR2, mediate murine sickle cell vaso-occlusion during hemolytic transfusion reactions

Post-Transfusion Hyperhaemolysis in a Patient with Sickle Cell Disease: Use of Steroids and Intravenous Immunoglobulin to Prevent Further Red Cell Destruction

A simple, practical model for reducing alloimmunization in patients with sickle cell disease

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