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Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevier Inc. Some material was previously published. 1 Chapter 13 Chapter 13 Hemolytic Disease of Hemolytic Disease of the Fetus and Newborn the Fetus and Newborn

Ppt hemolytic disease of the newborn & fetus

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Page 1: Ppt hemolytic disease of the newborn & fetus

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1

Chapter 13Chapter 13

Hemolytic Disease of the Fetus Hemolytic Disease of the Fetus and Newbornand Newborn

Page 2: Ppt hemolytic disease of the newborn & fetus

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Hemolytic Disease of the Fetus Hemolytic Disease of the Fetus and Newborn (HDFN)and Newborn (HDFN)

A disorder of the fetus or newborn in which A disorder of the fetus or newborn in which fetal red blood cells are destroyed by fetal red blood cells are destroyed by maternal IgG antibodiesmaternal IgG antibodies

The IgG antibodies cross the placenta and The IgG antibodies cross the placenta and shorten red cell survivalshorten red cell survival

The premature red cell destruction results The premature red cell destruction results in disease varying from mild anemia to in disease varying from mild anemia to death in uterodeath in utero

Page 3: Ppt hemolytic disease of the newborn & fetus

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Etiology in FetusEtiology in Fetus

The mother is exposed during first The mother is exposed during first pregnancypregnancy

In a subsequent pregnancy, IgG In a subsequent pregnancy, IgG antibodies cross the placentaantibodies cross the placenta

The antigens bind to fetal cells and red The antigens bind to fetal cells and red cells are destroyed, liberating hemoglobincells are destroyed, liberating hemoglobin

The hemoglobin is metabolized to indirect The hemoglobin is metabolized to indirect hemoglobinhemoglobin

Anemia occurs, which can lead to deathAnemia occurs, which can lead to death

Page 4: Ppt hemolytic disease of the newborn & fetus

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Etiology in NewbornEtiology in Newborn

After delivery, red cell destruction continuesAfter delivery, red cell destruction continues Indirect bilirubin is still being releasedIndirect bilirubin is still being released Newborn liver does not produce glucuronyl Newborn liver does not produce glucuronyl

transferase to process the indirect bilirubintransferase to process the indirect bilirubin High indirect bilirubin levels result in High indirect bilirubin levels result in

jaundice and also can cause brain damage, jaundice and also can cause brain damage, deafness, mental retardation, or death deafness, mental retardation, or death

Page 5: Ppt hemolytic disease of the newborn & fetus

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Figure 13-1Figure 13-1

From Ortho Diagnostics: Blood group antigens and antibodies as applied to hemolytic disease of the fetus and newborn, Raritan, NJ, 1968, Ortho Diagnostics.

Page 6: Ppt hemolytic disease of the newborn & fetus

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Rh HDFNRh HDFN

Anti-D responsible for most severe cases Anti-D responsible for most severe cases of HDFN of HDFN

Alloimmunization occurs during first Alloimmunization occurs during first pregnancy if baby is D-positive but rarely pregnancy if baby is D-positive but rarely results in clinical symptomsresults in clinical symptoms

Following antibody production, subsequent Following antibody production, subsequent pregnancies with a D-positive infant are pregnancies with a D-positive infant are affected to varying degreesaffected to varying degrees

Rh immune globulin (RhIG), introduced in Rh immune globulin (RhIG), introduced in 1968, dramatically reduced the incidence 1968, dramatically reduced the incidence of Rh HDFNof Rh HDFN

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ABO HDFNABO HDFN

More common, but most cases are More common, but most cases are subclinical and do not need treatmentsubclinical and do not need treatment

If jaundice develops, mild cases can be If jaundice develops, mild cases can be treated by phototherapytreated by phototherapy

Usually group O mothers with group A or B Usually group O mothers with group A or B infantsinfants

Unlike D, can affect first pregnancyUnlike D, can affect first pregnancy

Page 8: Ppt hemolytic disease of the newborn & fetus

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Alloantibodies Causing HDFNAlloantibodies Causing HDFN

Any IgG antibody is capable of causing Any IgG antibody is capable of causing HDFN if the fetal red cells possess HDFN if the fetal red cells possess antigens that the mother lacksantigens that the mother lacks

Anti-c is the second most common cause Anti-c is the second most common cause of HDFN; can be in combination with anti-of HDFN; can be in combination with anti-DD

Next is anti-KNext is anti-K

Page 9: Ppt hemolytic disease of the newborn & fetus

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Table 13-1Table 13-1

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Prediction of HDFNPrediction of HDFN

Prenatal testing should be donePrenatal testing should be done D-negative mothers need to be identifiedD-negative mothers need to be identified Mothers who have antibodies capable of Mothers who have antibodies capable of

causing HDFN need to be identifiedcausing HDFN need to be identified Maternal history is also importantMaternal history is also important

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Box 13-1Box 13-1

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Antibody TitrationAntibody Titration

Commonly used to predict severity of HDFNCommonly used to predict severity of HDFN Titer done early in pregnancy Titer done early in pregnancy Repeated at 16 to 22 weeks and repeated Repeated at 16 to 22 weeks and repeated

at 1- to 4-week intervalsat 1- to 4-week intervals A twofold rise in titer is an indication for A twofold rise in titer is an indication for

further monitoringfurther monitoring To ensure significance, titers should be To ensure significance, titers should be

done using the same method and cellsdone using the same method and cells

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AmniocentesisAmniocentesis

Measurement of bilirubin in amniotic fluid Measurement of bilirubin in amniotic fluid can help indicate level of red cell can help indicate level of red cell destructiondestruction

Aspirated fluid is scanned on a Aspirated fluid is scanned on a spectrophotometer from 350 to 700 nm. spectrophotometer from 350 to 700 nm. The change in optical density above the The change in optical density above the baseline 450 nm is plotted on a Liley graphbaseline 450 nm is plotted on a Liley graph

Page 14: Ppt hemolytic disease of the newborn & fetus

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Figure 13-2Figure 13-2

From Mollison PL, Engelfriet CP, Conteras M: Blood transfusion in clinical medicine, ed 9, London, 1993, Blackwell Science.

Page 15: Ppt hemolytic disease of the newborn & fetus

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Liley GraphLiley Graph

The Liley graph defines three zones to The Liley graph defines three zones to estimate severity:estimate severity:

Correlates with severe if results are in the top zone Correlates with severe if results are in the top zone Mid zone correlates with moderate Mid zone correlates with moderate Bottom zone correlates with mild HDFNBottom zone correlates with mild HDFN

Page 16: Ppt hemolytic disease of the newborn & fetus

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Interpretation of Liley GraphInterpretation of Liley Graph

Based on amniotic fluid analysisBased on amniotic fluid analysis Three alternatives exist:Three alternatives exist:

- Allow pregnancy to continue- Allow pregnancy to continue

- Perform intrauterine transfusion- Perform intrauterine transfusion

- Induce labor- Induce labor If labor is to be induced, L/S ratio needs to If labor is to be induced, L/S ratio needs to

be done to determine fetal lung maturitybe done to determine fetal lung maturity

Page 17: Ppt hemolytic disease of the newborn & fetus

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Postpartum TestingPostpartum Testing

Collect sample of cord bloodCollect sample of cord blood Sample should be labeled and stored up to Sample should be labeled and stored up to

7 days7 days All infants born to D-negative mothers All infants born to D-negative mothers

should be tested for D, including weak Dshould be tested for D, including weak D D-negative mothers of D-positive infants, D-negative mothers of D-positive infants,

including weak D-positive infants, are including weak D-positive infants, are candidates for RhIG candidates for RhIG

Page 18: Ppt hemolytic disease of the newborn & fetus

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Testing on Infants with Suspected Testing on Infants with Suspected HDFNHDFN

A diagnosis is based on medical history, A diagnosis is based on medical history, physical examination, and lab testing on physical examination, and lab testing on mother and infantmother and infant

Tests to be performed:Tests to be performed: ABO only forward typeABO only forward type D testingD testing Direct antiglobulin test if positive elution is done Direct antiglobulin test if positive elution is done Eluate should be tested against A, B, and O cellsEluate should be tested against A, B, and O cells Negative with all cells, low-frequency antibodyNegative with all cells, low-frequency antibody

Page 19: Ppt hemolytic disease of the newborn & fetus

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Prevention of HDFNPrevention of HDFN

RhIG provides protection for 30 mL of fetal whole RhIG provides protection for 30 mL of fetal whole bloodblood

A 300-A 300-µg dose is routinely administered to D-µg dose is routinely administered to D-negative mothers antepartum at 28 weeks’ negative mothers antepartum at 28 weeks’ gestation gestation

Also administered to D-negative mothers after Also administered to D-negative mothers after amniocentesis, abortion, termination of ectopic amniocentesis, abortion, termination of ectopic pregnancy, chorionic villus sampling, percutaneous pregnancy, chorionic villus sampling, percutaneous umbilical blood sampling (PUBS), or abdominal umbilical blood sampling (PUBS), or abdominal traumatrauma

Page 20: Ppt hemolytic disease of the newborn & fetus

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Postpartum AdministrationPostpartum Administration

Cord blood from all infants born to D-Cord blood from all infants born to D-negative women should be tested for D negative women should be tested for D antigenantigen

Any D-negative woman who delivers a D- Any D-negative woman who delivers a D- positive infant is given a full dose of RhIG positive infant is given a full dose of RhIG within 72 hours of delivery within 72 hours of delivery

There should be a history of negative There should be a history of negative antibody screen during the current antibody screen during the current pregnancy pregnancy

Page 21: Ppt hemolytic disease of the newborn & fetus

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Screening for Fetomaternal Screening for Fetomaternal Hemorrhage (FMH)Hemorrhage (FMH)

If during delivery a woman exceeds an If during delivery a woman exceeds an FMH greater than 30 mL of D-positive fetal FMH greater than 30 mL of D-positive fetal cells, it is essential that she receive more cells, it is essential that she receive more than one dose of RhIGthan one dose of RhIG

Currently most frequently used method to Currently most frequently used method to screen for FMH is the rosette testscreen for FMH is the rosette test

Page 22: Ppt hemolytic disease of the newborn & fetus

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Figure 13-4Figure 13-4

Courtesy Gamma Biologicals, Houston.

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Quantifying FMHQuantifying FMH

Kleihauer-Betke acid elution is the method most Kleihauer-Betke acid elution is the method most frequently used to quantify the number of fetal frequently used to quantify the number of fetal cells in the mother’s circulationcells in the mother’s circulation

Page 24: Ppt hemolytic disease of the newborn & fetus

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Figure 13-5Figure 13-5

Page 25: Ppt hemolytic disease of the newborn & fetus

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Treatment of HDFNTreatment of HDFN

In utero treatment includes intrauterine In utero treatment includes intrauterine transfusions to correct anemiatransfusions to correct anemia

Recently PUBS has been used to transfuse Recently PUBS has been used to transfuse directly into the umbilical veindirectly into the umbilical vein

Blood for intrauterine transfusion should be group Blood for intrauterine transfusion should be group O, D-negative RBCs less than 7 days old, O, D-negative RBCs less than 7 days old, irradiated, cytomegalovirus (CMV) negative, and irradiated, cytomegalovirus (CMV) negative, and hemoglobin S negativehemoglobin S negative

Donor cells are crossmatched with mother’s Donor cells are crossmatched with mother’s serumserum

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Postpartum TreatmentPostpartum Treatment

Phototherapy: Infants are exposed to blue Phototherapy: Infants are exposed to blue light in the 420- to 475-nm rangelight in the 420- to 475-nm range

If infant fails to respond, exchange If infant fails to respond, exchange transfusion is neededtransfusion is needed

For exchange transfusion, ABO and D For exchange transfusion, ABO and D testing is done on infant. Mother’s plasma testing is done on infant. Mother’s plasma is used for antibody screenis used for antibody screen

CMV and hemoglobin S negative blood CMV and hemoglobin S negative blood used used

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Box 13-5Box 13-5