F etus NAHLA BAKHAMIS 10152015 NAHLA BAKHAMIS MSc 1 Haemolytic Disease RBC destruction in vivo may associated with anaemia Intracellularextracellular haemolysis 10152015 NAHLA BAKHAMIS MSc ID: 301753
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Haemolytic Disease of the New-born and Fetus
NAHLA BAKHAMIS
10/15/2015
NAHLA BAKHAMIS, MSc
1Slide2
Haemolytic Disease
RBC destruction in vivo
may associated with anaemia
Intracellular/extracellular haemolysis
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Intracellular haemolysis
RBC destructed inside the blood vessels
mediated by
complement activation
result in free Hb circulation -- is toxic to the kidney & DIC
Hb immediately bound to haptoglobin in the plasma
Hb-haptoglobin
complex removed by hepatic RE cells
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Extracellular haemolysis
RBC destructed in the RES (liver & spleen)
macrophage mediated
free Hb --- unconjugated bilirubin
transfer to the liver --- conjugated to glucuronic acid – removed in faeces
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Fetal Liver
Can’t metabolize bilirubin
causing kidney failure and brain tissue damage
newborn
liver enzymes are not functioning before 10 days post delivery
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HDN-HDF
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HDN-HDF
Triggered by maternal Abs to paternally inherited Ag in the
fetus
RhD most frequently
Fetal RhD
+ RBCs --- maternal circulation FMH mother immune system form anti D Abs for many years
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First pregnancy
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HDN-HDF
Second pregnancy: maternal IgG anti-D sensitize the D+
fetal cells
destroyed by macrophages in RES
cause sever anaemia erythroblastosis
fetalis sever oedema of
fetal
liver and spleen
in severe cases
Hydrops
fetalis
--- intrauterine death & still birth
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Second pregnancy
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Erythroblastosis fetalis
clinical findings: - anaemia < 15 gm/dl
- jaundice
- immature RBCs 10/15/2015
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Increased no. of immature RBCsSlide12
HDN-HDF
During pregnancy; excess free bilirubin --- transport to mother --- metabolism & excretion
At delivery; maternal system no longer removing bilirubin + neonatal liver not functioning
When unconjugated (indirect) bilirubin reach toxic leve
l (18-20 mg/dl)
Free bilirubin damage the brain Kernicterus due to sever jaundice
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Clinical features
mild form: mild anaemia
sever form: kernicterus
intrauterine death (hydrops fetalis):
pathophysiology :
- extravascular haemolyse with extramedullary erythropoiesis
- hepatic & cardiac failure
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Clinical features
before birth:
Anaemia, heart failure, hydrops fetalis
After birth:
anaemia, heart failure, build up of bilirubin, kernicterus and sever growth retardation
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Blood film of HDF, showing
polychromasia
and increased number of normoplast Slide17
HD due to ABO incompatibility
very frequent but less sever
group O mother carrying A or B fetus
doesn’t cause problem to the fetus
in the uterus post delivery; jaundice and mild anaemia
can occur during 1
st
pregnancy
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HD due to ABO incompatibility
ABO HDN/F is less sever than Rh HDN/F because:
ABO Abs are mostly IgM
Neonatal RBCs express A and B poorly Many cells other than RBCs express Ag A and B thus absorb some of transferred Abs
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HD due to ABO incompatibility
Treatment
about 10% of cases phototherapy
administration of immunoglobulin
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HD due to Rh incompatibility
maternal anti D (most common cause & most severe)
mother (d/d) and child D/D or D/d
followed by anti c
first child --- safe
subsequent offspring that are Rh + will be affected
feto
-maternal haemorrhage
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Feto-maternal haemorrhage
placental membrane rupture 7%
abdominal trauma
abortion
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HD due to other blood groups incompatibility
can cause due to anti K,
Fy and other IgG Abs
maternal K suppress fetal erythropoiesis causing sever anaemia
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Antenatal testing
Screening test for pregnant woman:
ABO grouping
Rh grouping (test for weak D if initially –ve)
Antibody screen (to detect IgG, if –ve repeat at 24 or 28 weeks)
Antibody identification Titration of significant RBCs Abs
Paternal RBC typing
Fetal
amniocentesis and Doppler technique
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Kleihauer test for fetal RBCs
acid elution method:
adult Hb is eluted by acidic PH and appear as ghost cells
whereas fetal Hb
resist acidic PH and stain red other method include flow cytometry
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Amniocentesis and Cordocentesis
can be detected as early as 10-12 weeks gestation detect the gene for D Ag and any other Ag
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Treatment
Before birth:
Interauterin transfusion
Early delivery After birth
1. Phototherapy -change unconjugated bilirubin
- may avoid the need for exchange transfusion10/15/2015
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Treatment
2. New-born exchange transfusion:
small aliquots of blood
corrects anaemia
removal of bilirubin
removal of sensitized RBCs and Abs
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Selection of blood exchange transfusion
Group O RBCs
Rh –ve
units for Rh –ve cases
whole blood group O
blood less than 7 days old Irradiated
CMV negative
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Prevention (RhoGAM)
RhIg (RhoGAM) given to the mother to prevent immunization to D Ag
1 dose at 28 weeks gestation
RhIg attaches to
fetal RBCs in maternal circulation then removed in maternal spleen (no alloimmunization)
2
nd
dose 72 hrs after birth of Rh+ infant
also administration following termination of any pregnancy, and following accidental transfusion with Rh+ RBCs
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Summary
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HDN/F occurs when IgG antibodies produced by the mother against the corresponding
Ags which is absent in her, crosses the placenta and destroy
fetal RBCs
Proper early management of Rh-HDN saves lives of child and future pregnancies
ABO-HDN is usually mild
other blood group
Ags
can also cause HDN (anti-c and anti-K)Slide37
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Questions ??