Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women.
Adult ; Blood Group Antigens/immunology ; *Blood Group Incompatibility ; Diseases in Twins/diagnosis/*immunology ; Erythroblastosis, Fetal/*diagnosis/immunology ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Isoantigens/immunology ; Jaundice, Neonatal/complications/immunology/therapy ; Male ; Phenotype ; Phototherapy ; Pregnancy ; Pregnancy Complications, Hematologic/diagnosis/*immunology ; Twins

Here we report a severe case of hemolytic anemia of the newborn with kernicterus caused by anti-Di(a) antibody. A full term male infant was transferred due to hyperbilirubinemia on the third day of life. Despite single phototherapy, the baby's total bilirubin had elevated to 30.1 mg/dL. After exchange transfusion, total bilirubin decreased to 11.45 mg/dL. The direct antiglobulin test on the infant's red cells was positive. The maternal and infant's sera showed a negative reaction in routine antibody detection tests, but were positive in Di(a) panel cells. The frequency of the Di(a) antigen among the Korean population is estimated to be 6.4-14.5%. Anti-Di(a) antibody could cause a hemolytic reaction against transfusion or hemolytic disease of the newborn. We suggest the need for reagent red blood cell panels to include Di(a) antigen positive cells in antibody identification test for Korean.
Alleles ; Bilirubin/blood ; Erythroblastosis, Fetal/*diagnosis/*immunology ; Humans ; Infant, Newborn ; Isoantibodies/*analysis ; Male ; Polymerase Chain Reaction ; Rh-Hr Blood-Group System/*analysis/blood

OBJECTIVETo report a rare case of hemolytic disease of the newborn (HDN) with kernicterus caused by anti-Di(a) diagnosed using high-throughput genotyping multiplex ligation-dependent probe amplification (MLPA).

METHODSConventional serological methods were used to detect the antibodies related with HDN. The genotypes of more than 40 red blood cell antigens for the newborn and her parents were obtained using the high-throughput MLPA assay. The antibody titers were tested using a standard serological method.

RESULTSThe unknown antibody against the low-frequency antigens was predicted based on the primary serological tests. The genotyping results for more than 40 red blood cell antigens of the newborn and her parents showed incompatible antigens of MNS and Diego blood group system, indicating the existence of anti-N or anti-Di(a). Further serological tests confirmed anti-Di(a) existence in the plasma of the newborn and her mother. The titer of anti-Di(a) in the mother's plasma was 1:32.

CONCLUSIONSevere HDN including kernicterus can result from anti-Di(a). High-throughput genotyping MLPA assay can help type some rare antigens in complicated cases. The reagent red cell panels including Di(a)-positive cells are necessary in routine antibody screening test in Chinese population.

Blood Group Incompatibility ; genetics ; Erythroblastosis, Fetal ; diagnosis ; immunology ; Exchange Transfusion, Whole Blood ; Female ; Genotype ; Humans ; Infant, Newborn ; Nucleic Acid Amplification Techniques ; methods ; Rh-Hr Blood-Group System ; genetics ; immunology ; Rho(D) Immune Globulin ; genetics ; immunology

OBJECTIVEAnti-D IgG in RhD negative pregnant women is the main antibody of Rh-induced hemolytic disease of newborn (HDN). The study aimed to investigate the clinical significance of anti-D IgG screening and titer detection in RhD negative pregnant women.

METHODSSera of 286 RhD negative pregnant women were collected. Microtube column indirect antiglobulin test was used to screen and identify anti-D IgG. The indirect antiglobulin test was used to test the titer of anti-D IgG.

RESULTSAnti-D IgG was identified in 21 cases (7.3%). The titer of anti-D showed an increasing trend with pregnancy progresses. The clinical outcomes of 12 fetuses (newborns) from positive anti-D pregnant women were observed. Two cases died in utero, 2 cases did not show abnormality and 8 cases had hemolysis. The 8 cases with hemolysis were treated with exchange transfusion or blood transfusion, and they had a good prognosis.

CONCLUSIONSThe screening and titer detection of anti-D IgG in RhD negative pregnant women are valuable in the prediction and treatment of HDN.

ABO Blood-Group System ; immunology ; Adult ; Blood Group Incompatibility ; Erythroblastosis, Fetal ; diagnosis ; Female ; Humans ; Immunoglobulin G ; blood ; Isoantibodies ; blood ; Pregnancy ; Rh-Hr Blood-Group System ; blood ; Rho(D) Immune Globulin

ABO Blood-Group System ; immunology ; Drugs, Chinese Herbal ; therapeutic use ; Erythroblastosis, Fetal ; classification ; prevention & control ; Female ; Humans ; Hyperbilirubinemia ; prevention & control ; Immunoglobulin G ; blood ; Infant, Newborn ; Phytotherapy ; Pregnancy ; Prenatal Diagnosis