Fatal hemolytic disease of the fetus and newborn associated with anti-Jra

Authors

  • Thierry Peyrard,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Bach-Nga Pham,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Lionel Arnaud,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Sophie Fleutiaux,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Yves Brossard,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Bénédicte Guerin,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Isabelle Desmoulins,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Philippe Rouger,

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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  • Pierre-Yves Le Pennec

    1. From the National Reference Laboratory for Blood Groups, the National Institute of Blood Transfusion, the National Institute for Health and Medical Research, INSERM Unit 665, National Institute of Blood, and the National Reference Center of Perinatal Hemobiology, Saint-Antoine Hospital, Paris; the French Blood Establishment Aquitaine-Limousin, Immunohematology Laboratory, and the Department of Pediatrics, Hospital of Pau, Pau; and the Immunohematology Laboratory, French Blood Establishment Aquitaine-Limousin, Biarritz, France.
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Thierry Peyrard, PharmD, MS, EurClinChem, National Reference Laboratory for Blood Groups, National Institute of Blood Transfusion, INSERM U665, 20 rue Bouvier, BP 79, 75522 Paris Cedex 11, France; e-mail: tpeyrard@ints.fr.

Abstract

BACKGROUND: Jra is a high-prevalence red cell (RBC) antigen. The clinical significance of anti-Jra is controversial. When hemolytic disease of the fetus and newborn (HDFN) occurred, most reported cases were clinically mild. We report the first case of fatal HDFN due to anti-Jra.

CASE REPORT: A 28-year-old Caucasian woman with transfusion history was monitored at the 29th week of pregnancy (G4P1). An ultrasound scan showed fetal cardiomegaly and hepatomegaly. An antibody directed against a high-prevalence antigen was detected, but without conclusive identification. An emergency cesarean section was performed at the 36th week. The newborn was hydropic and showed severe anemia. Death occurred 30 hours after birth.

MATERIALS AND METHODS: Serologic methods were performed to investigate the mother's RBCs and serum. An in vitro functional cellular assay and semiquantitative measurement of anti-Jra were used to determine the clinical significance of the antibody.

RESULTS: Anti-Jra was identified in the serum and Jr(a−) phenotype was confirmed. The anti-Jra titer was 1024, with predominant immunoglobulin (Ig)G1 and minor IgG4 subclasses. The functional cellular assay was consistent with an antibody unlikely to cause HDFN. Semiquantitative measurement of anti-Jra showed a reactivity equivalent to a 25 IU per mL (5 µg/mL) concentration of anti-D, a value associated with a significant risk of HDFN.

CONCLUSION: This is the first documented case of fatal HDFN due to anti-Jra. Therefore, we recommend close monitoring of pregnant women with a high-titer anti-Jra, especially those with an incompatible transfusion history and/or multiple pregnancies. This case report provides new arguments about the clinical significance of anti-Jra in the transfusion setting.

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