Cases reported "Erythroblastosis, Fetal"

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1/58. Use of recombinant human erythropoietin (EPO-alfa) in a mother alloimmunized to the Js(b) antigen.

    erythropoietin (EPO) is a glycoprotein hormone and the principal regulator of erythropoiesis in the fetus, newborn, and adult. EPO-alfa is erythropoietin manufactured by recombinant human dna technology (rhEPO). After counseling, a pregnant woman with anti-Js(b) in her serum was started on rhEPO (600 U/Kg, biweekly) to prevent anemia secondary to serial donations of her blood for fetal transfusions. After a total of 25 rhEPO infusions and autologous donation of 8 units of whole blood, maternal hemoglobin prior to the elective cesarean section at 37 weeks was 11.3 gm/dL. serum EPO concentration was determined in paired maternal and fetal blood samples, before ultrasound guided intravascular transfusions, in this alloimmunized Js(b)-negative and another Rh(D) alloimmunized pregnancy to determine possible correlations between maternal and fetal serum EPO. rhEPO prevented anemia in a patient who donated 8 units of blood from 18-37 weeks of pregnancy without inducing adverse biological effects such as hypertension or thrombotic complications in the placenta. Data presented in this study suggest that EPO does not cross the human placenta.
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2/58. Hemolytic transfusion reaction due to anti-Tc(a).

    BACKGROUND: Anti-Tc(a) detects a high-incidence antigen in the Cromer blood group system. Cromer system antibodies have not usually been associated with hemolytic transfusion reactions or hemolytic disease of the newborn. CASE REPORT: Anti-Tc(a) (initially identified in the patient's serum in 1982) was not detected when she was admitted to the hospital with upper gastrointestinal. bleeding. Three units of red cells were administered. The patient was discharged, but was readmitted to the hospital after her hemoglobin fell to 7.1 g per dL. Antibody detection tests remained negative and three additional units were transfused. Over the next 7 days, her hemoglobin steadily fell to 5.5 g per dL. The level of lactate dehydrogenase rose to 1257, the plasma hemoglobin rose to >16 mg per dL, and the haptoglobin decreased to <6 mg per dL. Five days after transfusion, her direct antiglobulin test was weakly reactive with complement-specific antiglobulin reagents. Eluates were nonreactive. Anti-Tc(a) was detected in her serum; no other antibodies were detected. Differential typing failed to detect any circulating Tc(a ) red cells. The antibody was strongly reactive in a monocyte monolayer assay. CONCLUSION: Although Cromer system antibodies have generally not been proven to be clinically significant in transfusion therapy, the destruction of red cells from six units of transfused Tc(a ) red cells in this patient indicates that anti-Tc(a) may have destructive potential in some patients.
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3/58. Use of a PCR-based assay for fetal Cw antigen genotyping in a patient with a history of moderately severe hemolytic disease of the newborn due to anti-Cw.

    Anti-Cw is an uncommon cause of clinically significant hemolytic disease of the newborn (HDN). We report an unusually severe case of HDN due to anti-Cw that required phototherapy and exchange transfusion. We also describe a novel PCR-RFLP method for Cw typing of fetal genomic dna that was used for prenatal diagnosis in a subsequent pregnancy. Following PCR amplification of a 163 bp segment of the RHCE gene containing the nucleotide 122 G to A substitution that corresponds to the Cw allele, Cw types were distinguished by TaqI digestion. PCR-RFLP analysis confirmed that the father and previously affected child were Cw-positive. The fetus was Cw-negative, thus excluding HDN in the current pregnancy and obviating the need for further invasive or noninvasive diagnostic procedures for the remainder of the pregnancy. This case illustrates the utility of PCR-based fetal genotype determination in pregnancies at risk of HDN due to uncommon red cell antibodies such as anti-Cw.
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4/58. Hemolytic disease of the newborn due to anti S antibodies.

    We report a case of hemolytic disease in a newborn due to anti S antibodies. Baby R was born at term to an O mother whose antibody screen was positive for phenotype big S. Cord blood eluate revealed anti-S RBC; antigen: RBC typing for S- was positive. physical examination of baby was unremarkable. The infant's HCT was 44.2 at 6 hours of age. At 48 hours, the HCT decreased to 33.5, bilirubin peaked to 5.4, retic had peaked to 6.8. By seven days, all these values reverted to the normal, and baby has remained asymptomatic.
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5/58. Subclinical hemolytic disease of the newborn due to anti-e.

    A case of subclinical hemolytic disease of the newborn resulting in reticulocytosis and positive direct and indirect antiglobulin tests in an infant both ABO- and Rh(D)-compatible with its mother is reported. The antibody was an IgG, non-complement-binding molecule present in low titer with a low avidity for the antigen, acted optimally in enzyme systems, did not manifest dosage effect, and caused slight extravascular destruction of fetal erythrocytes. Routine antibody screening of all pregnant women should be performed and neonatal cord blood specimens analyzed to avoid unexpected infant morbidity and mortality, and to characterize hemolytic disease of the newborn due to rare atypical antibodies more fully.
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6/58. First example of hemolytic disease of the newborn caused by anti-Or and confirmation of the molecular basis of Or.

    BACKGROUND AND OBJECTIVES: The rare MNS antigen Or (MNS31) is sensitive to ficin, papain and sialidase, but partially resistant to trypsin (0.05%); the effect of alpha-chymotrypsin is not known. A point mutation, 204C --> T in exon 3 of GYPA, is associated with the Or phenotype. We report here the first case of hemolytic disease of the newborn (HDN) caused by anti-Or, and expand the information on the nature of the Or determinant. MATERIALS AND methods: A woman, gravida 4, para 0, delivered a baby whose red blood cells (RBCs) were positive (2 ) on the direct antiglobulin test (DAT). The mother's serum, an eluate made from the baby's RBCs and the RBCs of the baby's father were investigated. Exon 3 of GYPA, extracted from the father's genomic dna, was amplified and sequenced. RESULTS: The mother's serum reacted at room temperature, 37 degrees C and on the indirect antiglobulin test with RBCs from the baby's father. The father's RBCs were M N S-s Or . The antibody in the mother's serum and in the baby's eluate was identified as anti-Or. The serum did not react with the father's RBCs treated with trypsin (180,000 U/ml), but did react with his alpha-chymotrypsin-treated RBCs. Amplification and sequencing of dna from the father revealed a single point mutation, 204C --> T, in GYPA exon 3. At birth, the baby had clinical symptoms of HDN and was transfused with 36 ml of packed RBCs and received phototherapy for eight days. At week 11, the baby's M N S s Or RBCs were negative on the DAT. CONCLUSION: This is the first case of HDN caused by anti-Or. The observed point mutation, 204C --> T, confirms that of a previous report and predicts a change of Arg (Or-) to Trp (Or ) at amino acid 31.
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7/58. Treatment with plasmapheresis and intravenous immunoglobulin in pregnancies complicated with anti-PP1Pk or anti-K immunization: a report of two patients.

    BACKGROUND AND OBJECTIVES: In addition to anti-D alloantibody, other antibodies such as anti-K antibody and anti-PP1Pk antibody have been reported to cause severe haemolytic disease of the newborn (HDN). HDN caused by anti-K results not only from destruction of red cells but also from suppression of erythropoiesis. Anti-PP1Pk has been associated with abortion early in pregnancy. We report on two patients, one with anti-PP1Pk and the other with anti-K, who were treated with plasmapheresis and intravenous immunoglobulin (IVIG) during pregnancy in an attempt to reduce the plasma antibody levels. MATERIALS AND methods: The patient with anti-PP1Pk had lost all seven previous fetuses in the first trimester and therefore therapy in this patient was started at 8 weeks of gestation. The second patient had been sensitized to the K antigen through blood transfusion and had had two intrauterine fetal deaths at 26 weeks of gestation with signs of hydrops fetalis. Treatment in this patient was started during the 16th week of pregnancy. RESULTS: As a result of therapy, the antibody titre was reduced in both patients. In the first patient a healthy infant was delivered by Caesarean section at 37 weeks of gestation. The second patient gave birth at 36 weeks of gestation. Neither newborn required exchange transfusion. CONCLUSION: In our two patients, plasmapheresis combined with IVIG proved successful in the management of fetomaternal incompatibilities where the mechanism of fetal loss differs from the classical anti-D.
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8/58. An unusual case of ABO-haemolytic disease of the newborn.

    Haemolytic disease of the newborn (HDN) is a clinical condition in which foetal red blood cells are destroyed by maternal alloantibodies directed against red cells antigens acquired from the father. These antibodies usually belong to the Rhesus (Rh) or ABO blood group systems. ABO-HDN is usually a sub-clinical condition and less severe than Rh-HDN. The placenta is relatively impermeable to naturally occurring IgM anti-A/anti-B antibodies. However, immune anti-A and anti-B of the IgG type will cross the placenta and may thus cause ABO-HDN. ABO-HDN is the commonest in Group O mothers having A infants. Occasionally it is seen in Group O or A (A2) mothers of Group B infants. The most severe disease is seen in immune anti-B rather than anti-A antibodies. There are at present no satisfactory methods to predict ABO-HDN in the antepartum period. We report here a case of ABO-HDN where mother who was B, Rh-positive; delivered a baby girl of A1B, Rh-positive who developed severe haemolytic disease. The baby was the third child. To our knowledge this is the first of this kind of severe ABO-HDN in bangladesh and one of the rarest ABO-HDN cases in the world.
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9/58. Hemolytic disease of the newborn due to isoimmunization with anti-E antibodies: a case report.

    Minor blood group hemolytic disease is extremely rare, since the overall potency of minor blood groups in inducing antibodies is significantly lower when compared with that of Rh (D) antigen. We hereby report a very rare case of severe neonatal anti-E hemolytic disease due to E minor blood group incompatibility. A term newborn born to a 27-year-old, gravida 3, para 3 mother was referred due to a high and increasing serum bilirubin level despite phototherapy on the 4th day of life. On admission physical examination was normal except for the jaundice, and results of the laboratory investigation demonstrated a moderate-to-severe anemia (hemoglobin 7.8 g/dl) and a severe hemolytic hyperbilirubinemia (serum total and indirect bilirubin levels 36 mg/ dl and 32.8 mg/dl, respectively; reticulocyte count 15%; and a positive direct antiglobulin test). As there was no apparent cause of the hemolytic disease such as Rh or ABO incompatibilities, further investigation (a positive indirect antiglobulin test and a positive irregular anti-E antibody in both the patient and mother, and minor blood group antigen profiles in family members compatible with E minor blood group isoimmunization) revealed the presence of anti-E hemolytic disease due to E minor blood group incompatibility. Two exchange transfusions with a 12-hour-interval were performed with minor blood group compatible fresh whole blood, and the patient was discharged in a healthy condition on the 10th postnatal day. If the most common causes of severe neonatal hemolytic disease such as Rh and ABO incompatibilities cannot be demonstrated in a newborn with significant hemolytic hyperbilirubinemia, anti-E hemolytic disease should strongly be considered in differential diagnosis. It should be kept in mind that a very severe from of minor group antibody hemolytic disease characterized by anemia and severe hyperbilirubinemia many exchange transfusions may be encountered during the course of the disease.
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10/58. Anti-Ce complicating two consecutive pregnancies with increasing severity of haemolytic disease of the newborn.

    The Ce antigen is expressed on red cells of individuals with Rh haplotypes, R1 or CDe and r' or Cde. However, anti-Ce is rare, and only two cases of severe haemolytic disease of the newborn (HDN) caused by this antibody have been described (Malde et al., 2000; Wagner et al., 2000). We describe a woman who was found to have anti-Ce in her second pregnancy, resulting in a neonate with HDN that required exchange transfusion. She subsequently had a twin pregnancy, where both the twins were affected by severe haemolytic disease (HD) of the fetus because of anti-Ce and required repeated fetal transfusions, followed by exchange transfusions after birth. This is the first reported case of HD caused by anti-Ce requiring fetal transfusions.
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