Cases reported "Erythroblastosis, Fetal"

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1/9. Haemolytic disease of the newborn due to anti-Dia and incidence of the Dia antigen in poland.

    A strong complement-fixing incomplete anti-Dia was found in the serum of a Polish mother who gave birth to a newborn suffering from a severe haemolytic anaemia. The whole family was of Polish origin. 9,661 donor blood samples from different regions of poland were tested against antiserum derived from that mother. Dia antigen was present on red cells of 45 (0.46%) individuals. All of them were of Polish origin. In some cases, family studies were undertaken.
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ranking = 1
keywords = anaemia
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2/9. ABO incompatibility due to immunoglobulin g anti-B antibodies presenting with severe fetal anaemia.

    ABO incompatibility is a common haematological problem affecting the newborn. The haemolysis is widely accepted to follow a relatively benign course rarely causing the escalating levels of hyperbilirubinaemia and significant anaemia associated with Rh haemolytic disease of the newborn. case reports of fetal hydrops secondary to ABO incompatibility are particularly rare. We describe two cases, first that of a twin pregnancy with both fetuses developing severe anaemia at 20 weeks gestation, and then a second case of a preterm baby demonstrating aggressive haemolysis and anaemia within hours of delivery. Both mothers were of black Africian origin and both were identified to have elevated titres of IgG anti-B antibodies.
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ranking = 7
keywords = anaemia
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3/9. Use of red cells preserved in extended storage media for exchange transfusion in anti-k haemolytic disease of the newborn.

    Anti-k is a Kell-related antibody. There is little correlation between the maternal antibody titre and the severity of haemolytic disease of the foetus and newborn, and anaemia is usually associated with low bilirubin levels. Severe erythroblastosis has been reported with a low titre anti-k (IAT 8-16). We report a case of severe haemolytic disease of the newborn (HDN) due to anti-k. HDN was associated with a normal bilirubin level and reticulocytopenia. The foetus was monitored by ultrasound, and delivery by elective caesarean section (CS) was planned. The mother was admitted 1 week before the expected date of delivery, and the infant was delivered by urgent CS. The infant required exchange transfusion. As suitable plasma-reduced (k antigen(-)) red cell units were not readily available, k- SAGM red cell units (preserved in extended storage media: SAGM sodium chloride, adenine, glucose and mannitol) were provided. The post-transfusion Hb remained stable, and the infant did not require further transfusion support. Our findings (reticulocytopenia and normal bilirubin levels) support the hypothesis that the pathogenesis of anaemia and haemolysis in anti-k HDN may be similar to that in anti-K (suppression of erythropoesis and immune destruction of K erythroid progenitor cells by macrophages in the foetal liver). The ideal product for exchange transfusion is plasma-reduced RBC, less than 5-days old. We provided a 4-day-old SAGM red cell unit for exchange transfusion in a term infant, and this was uneventful. Caution should be taken, however, and renal function and electrolyte levels should be monitored closely. More information is required regarding the safety of SAGM units for exchange transfusion.
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ranking = 2
keywords = anaemia
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4/9. Anti-D in a 'D positive' mother giving rise to severe haemolytic disease of the newborn. A dilemma in antenatal immunohaematological testing.

    An Rh positive woman having the Rh(D) variant designated DVI formed a potent anti-D which caused a severe haemolytic disease in the infant. The infant received three exchange transfusions within the first 21 hours after delivery and, because of a 'late anaemia', three additional blood transfusions during the first 3 months of life. The authors point out the distinction between D variant antigens, where certain epitopes of the D antigen are lacking, and qualitatively normal D antigens with a reduced reactivity, Du. women with D variant antigens should be subject to the same antenatal serological control as those who are Rh(D) negative, and if they form anti-D the infant should be controlled as in other cases where haemolytic disease of the newborn is suspected.
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ranking = 1
keywords = anaemia
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5/9. Sinusoidal antenatal fetal heart rate patterns.

    The sinusoidal fetal heart rate pattern is significantly associated with severe fetal anaemia when seen in the antenatal period. Strict adherence to definition is important, as pseudosinusoidal patterns do not have the same grave prognostic significance. If this fetal heart rate pattern is seen antenatally it is our contention that the baby should be delivered urgently by Caesarean section, unless the fetal anaemia can be treated in utero.
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ranking = 2
keywords = anaemia
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6/9. Direct intravascular fetal blood transfusion by fetoscopy in severe Rhesus isoimmunisation.

    Two fetuses with severe anaemia due to rhesus incompatibility each received two early blood transfusions (between 23 and 25 weeks) by fetoscopy. The blood was given directly into an umbilical vessel, either at the umbilicus or at the placental cord insertion. fetal blood samples were taken before and after transfusion to assess the haematological status of the fetus. One grossly hydropic fetus survived.
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ranking = 1
keywords = anaemia
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7/9. Haemolytic disease of the newborn due to Rhesus anti-e antibody.

    Haemolytic disease of the newborn due to rhesus anti-e alone is a rare occurrence. This condition is described in the second child of an R2R2 mother who had not previously been transfused. The antibody was of IgG subclasses IgG1 IgG3 and was detectable on the baby's cells for 4 months after birth. The anaemia was mild and persisted for the same period.
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ranking = 1
keywords = anaemia
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8/9. Three examples of Rh haemolytic disease of the newborn with a negative direct antiglobulin test.

    Typically the serological diagnosis of alloimmune haemolytic disease of the newborn (HDN) includes a positive direct antiglobulin test on the infant's red cells, and the presence of an IgG red cell alloantibody in both maternal and cord sera. HDN with a negative direct antiglobulin test has been reported with anti-A and anti-B, but not with other red-cell alloantibodies. In this report we describe four examples of HDN in infants whose red cells had a negative direct antiglobulin test. The first case was diagnosed retrospectively when the infant was admitted to hospital aged 3 weeks with severe anaemia and cardiac failure, and subsequently died. Maternal and infant sera were both shown to contain anti-C: however, the direct antiglobulin test on the infant's red cells was negative. Approximately 1 year later the mother of this infant gave birth to triplets: soon after birth one of the triplets required an exchange transfusion, one had hyperbilirubinaemia, and the third was unaffected. Anti-C and anti-e were detectable in the maternal serum at this time. The most probable Rh genotypes of the two affected infants were R1R2 (CDe/cDE), while the Rh genotype of the unaffected infant was R2R2 (cDE/cDE). Anti-c was implicated as causing HDN in a fourth infant (from a different family) who was a hydropic stillborn. The direct antiglobulin test on fetal blood was negative and other causes of non-immune hydrops were excluded. These four infants provide evidence that the direct antiglobulin test may be negative in some severely affected and even fatal cases of HDN.
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ranking = 1
keywords = anaemia
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9/9. A severe transfusion-dependent congenital dyserythropoietic anaemia presenting as hydrops fetalis.

    We describe a case of congenital dyserythropoietic anaemia (CDA) presenting at week 20 of pregnancy with hydrops fetalis and very severe anaemia. fetal blood examination showed marked erythroblastosis with morphologic alterations while the basic haematological and biochemical tests were in the normal range. The fetus received intravascular red blood cells transfusion at 20 and 25 weeks of gestation. After a caesarean section at week 33 the child became transfusion dependent. Haematological investigations demonstrated that the child is affected by a CDA-like picture which, although morphologically similar to CDA II, is Ham test negative and does not show the typical membrane proteins alterations.
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ranking = 6
keywords = anaemia
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