Partial C antigen in sickle cell disease patients: clinical relevance and prevention of alloimmunization

Tournamille, Christophe; Meunier-Costes, Natacha; Costes, Bruno; Martret, Jennifer; Barrault, Aurélie; Gauthier, Philippe; Galactéros, Frédéric; Nzouékou, Ruben; Bierling, Philippe

doi:10.1111/j.1537-2995.2009.02382.x

Cited by 67 publications

(71 citation statements)

References 16 publications

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“…A previous study showed that the C antigen should always be evaluated for a partial variant in SCD patients. 11 In patient 3, a complex combination of rare alleles was found but was not involved in the index DHTR. Finally, patient 8, who had a weak D type 4, developed anti-D antibodies after receiving a single D+ unit.…”

Section: Discussionmentioning

confidence: 99%

“…[5][6][7][8][9] DHTR has been reported to occur in 4-11% of SCD patients given blood transfusions. 6,[10][11][12] DHTRs occur from a few days to two weeks after a blood transfusion and manifest as clinical features of acute hemolysis (hemoglobinuria, jaundice, and pallor) combined with symptoms suggesting severe vaso-occlusive crisis (pain, fever, and sometimes acute chest syndrome). The destruction of both the donor's and the recipient's RBCs leads to an abrupt drop in the hemoglobin level, to a value lower than the pre-transfusion value.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Delayed hemolytic transfusion reaction in children with sickle cell disease

Montalembert¹,

Dumont²,

Heilbronner³

et al. 2011

Haematologica

106

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Delayed hemolytic transfusion reaction in children with sickle cell disease

Montalembert¹,

Dumont²,

Heilbronner³

et al. 2011

Haematologica

106

View full text Add to dashboard Cite

“…Although we cannot exclude that non-extended matching has been applied in some cases due to lack of time or limited supply in acute situations, these numbers do provide insight in the effectiveness of extended matching policies in daily practice. Alternatively, the substantial number of Rh antibodies found may also be explained by diversity in the RH locus in the SCD population [25,26]. Variant alleles encoding altered or partial Rh antigens, not detected by routine serologic testing, are highly prevalent in the population from African ancestry.…”

Section: Discussionmentioning

confidence: 99%

Early occurrence of red blood cell alloimmunization in patients with sickle cell disease

et al. 2016

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Red blood cell (RBC) alloimmunization is a major complication of transfusion therapy in sickle cell disease (SCD). Identification of high-risk patients is hampered by lack of studies that take the cumulative transfusion exposure into account. In this retrospective cohort study among previously non-transfused SCD patients in the Netherlands, we aimed to elucidate the association between the cumulative transfusion exposure, first alloimmunization and independent risk factors. A total of 245 patients received 11 952 RBC units. Alloimmunization occurred in 43 patients (18%), half of them formed their first alloantibody before the 8th unit. In patients with exposure to non-extended matched transfusions (ABO and RhD) the cumulative alloimmunization risk increased up to 35% after 60 transfused units. This was significantly higher compared to a general transfused population (HR 6.6, CI 4.2-10.6). Receiving the first transfusion after the age of 5 was an independent risk factor for alloimmunization (HR 2.3, CI 1.0-5.1). Incidental, episodic transfusions in comparison to chronic scheme transfusions (HR 2.3, CI 0.9-6.0), and exposure to non-extended matched units in comparison to extended matching (HR 2.0, CI 0.9-4.6) seemed to confer a higher alloimmunization risk. The majority of first alloantibodies are formed after minor transfusion exposure, substantiating suggestions of a responder phenotype in SCD and stressing the need for risk factor identification. In this study, older age at first transfusion, episodic transfusions and non-extended matched transfusions appeared to be risk factors for alloimmunization.

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“…5,6,23 Importantly, Rh antibodies continue to occur in patients who receive donor units that are antigen-matched by serology for Rh D, C, and E antigens because of RH variation at the genetic level. We previously reported that individuals who express only altered or partial Rh antigen (and no corresponding conventional protein) are at increased risk of alloimmunization.…”

Section: Discussionmentioning

confidence: 99%

Whole-exome sequencing for RH genotyping and alloimmunization risk in children with sickle cell anemia

et al. 2017

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Key Points• WES can be applied for precise RH genotyping, detection of new or uncommon variants, and determination of RHD zygosity.• An altered RH genotype is a risk factor for Rh alloimmunization in patients with sickle cell anemia.RH genes are highly polymorphic and encode the most complex of the 35 human blood group systems. This genetic diversity contributes to Rh alloimmunization in patients with sickle cell anemia (SCA) and is not avoided by serologic Rh-matched red cell transfusions.

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Partial C antigen in sickle cell disease patients: clinical relevance and prevention of alloimmunization

Abstract: This study demonstrates the need to detect partial C within C+ SCD patients and to prevent immunization. A larger number of Afro-Caribbeans donors is needed to provide these patients with C- RBCs.

Cited by 67 publications

References 16 publications

Delayed hemolytic transfusion reaction in children with sickle cell disease

Delayed hemolytic transfusion reaction in children with sickle cell disease

Early occurrence of red blood cell alloimmunization in patients with sickle cell disease

Whole-exome sequencing for RH genotyping and alloimmunization risk in children with sickle cell anemia

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