Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients' red cells

King, Karen E.; Shirey, R. Sue; Lankiewicz, Michael W.; Young-Ramsaran, Joy; Ness, Paul M.

doi:10.1046/j.1537-2995.1997.37497265337.x

Cited by 157 publications

(126 citation statements)

References 15 publications

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“…These cases included patients without alloantibodies and documented alloimmunized patients who received matched units for those target antigens. 96,97 The lack of detectable new antibodies in the post-transfusion screening test is consistent with a nonantibody-dependent mechanism of posttransfusion hemolysis as described in the previous section.…”

Section: Clinical and Biologic Characteristics Of The Antibodies In Dhtrsupporting

confidence: 82%

Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management

Yazdanbakhsh

Ware

Noizat‐Pirenne

2012

Blood

339

350

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Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease. Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions and difficulty in finding compatible units, which can cause transfusion delays. In this review, we discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger delayed hemolytic transfusion reactions in sickle cell disease, and we describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication. (Blood. 2012;120(3):528-537) IntroductionBlood transfusion remains a cornerstone of treatment of patients with sickle cell disease (SCD). Despite improved patient outcomes with hydroxyurea administration, indications for chronic transfusions have increased in the last 10 years and are associated with considerable reduction in morbidity and mortality, most notably in preventing first stroke in children. [1][2][3] However, transfusions can lead to erythrocyte alloimmunization with serious complications for the patient. These antibodies are often directed against antigens expressed on RBCs of white persons, which represent the majority of donors in Western countries. 4 Finding compatible units lacking those antigens can sometimes be difficult, and identifying and characterizing the antibodies can be timeconsuming and laborious, causing transfusion delays. Genetic as well as acquired patient-related factors are likely to influence the process of alloimmunization.The most serious consequence of alloimmunization in SCD patients is the risk of developing a delayed hemolytic transfusion reaction (DHTR), which can be life-threatening. In many cases of DHTR in SCD, the patient's hemoglobin level falls below the pretransfusion level, suggesting that, in addition to hemolysis of the transfused RBCs, the patient's own RBCs are lysed, a condition known as hyperhemolysis. Additional transfusions may exacerbate the hemolysis and further worsen the degree of anemia. The destruction of the patient's own RBCs in DHTR of SCD is partly explained by the presence of autoantibodies 5 because alloimmunization is known to trigger autoantibody production. However, DHTR/hyperhemolysis cases have also been reported in the absence of detectable alloantibodies or autoantibodies.In this review, we discuss the known risk factors associated with alloimmunization, then emphasize possible mechanisms that can trigger autoimmunization and DHTR in SCD, and finally describe the challenges in transfusion management of these patients. We emphasize opportunities and emerging approaches for minimizing this life-threatening complication. RBC alloimmunization pathobiologyAlloimmunization to erythrocytes involves multiple steps, including RBC antigen recognition, processing, and presentation of antigen by HLA class II to TCR, activation of CD4 ...

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Section: Clinical and Biologic Characteristics Of The Antibodies In Dhtrsupporting

confidence: 82%

Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management

Yazdanbakhsh

Ware

Noizat‐Pirenne

2012

Blood

339

350

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“…A second transfusion of group O RBC units, delivered in emergency due to the critical state of the patient, worsened the situation, leading to the death of the patient. In SCD patients, the physiopathology of DHTR seems to involve at least two mechanisms: 6,12 in addition to the restimulation of antibody production by transfusion, an additional mechanism, triggered by transfusion, probably operates, inducing or potentiating the hyperhemolysis syndrome. Indeed, in about 30% of cases, it is considered that no antibodies are involved.…”

Section: Discussionmentioning

confidence: 99%

Anti‐HI can cause a severe delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease patients

et al. 2016

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“…In other words, the destruction of autologous red cells is a sequela of the initial immune-mediated destruction of transfused red cells. In some ways, this is not dissimilar to the autologous destruction of platelets in posttransfusion purpura [3,42]. Autologous destruction of red cells in DHTR has previously been demonstrated Red cell transfusions both immediately and those after the acute episode of hyperhemolysis were type specific and undergone extended matching.…”

Section: Bystander Hemolysismentioning

confidence: 97%

“…One of the earliest theories is that of "bystander hemolysis" whereby destruction of autologous antigen-negative red cells occurs in parallel to that of antigen-positive red cells leading to a drop in Hb to below pretransfusion level [3]. In other words, the destruction of autologous red cells is a sequela of the initial immune-mediated destruction of transfused red cells.…”

Section: Bystander Hemolysismentioning

confidence: 99%

Hyperhemolysis in Patients With Hemoglobinopathies: A Single-Center Experience and Review of the Literature

Danaee

Inusa

Howard

et al. 2015

Transfusion Medicine Reviews

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Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients' red cells

Abstract: Hemoglobin S provides a biologic marker for monitoring autologous red cell loss in sickle cell patients. We have shown one patient with clinical evidence of bystander hemolysis complicating a DHTR.

Cited by 157 publications

References 15 publications

Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management

Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management

Anti‐HI can cause a severe delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease patients

Hyperhemolysis in Patients With Hemoglobinopathies: A Single-Center Experience and Review of the Literature

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