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7/31/2019 Hemolytic Disease
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HEMOLYTIC DISEASE OFTHE NEWBORN (HDN)
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Objective
Definition
State the etiology and pathogenesis of HDN
State the prevention of HDN including the use of
Rh immunoglobin List down the antenatal and post natal tests for
HDN
Select the compatible blood for exchange
transfusion Describe how the compatibility testing for
exchange transfusion is done
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Content
Definition of HDN
Classification
Etiology and pathogenesis of HDN
Laboratory investigation
Neonatal test
Maternal blood test
Exchange transfusion
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Definition
Also known as erythroblastosis fetalis(presence of nucleated RBCs), or hydropsfetalis (edema)
Is a condition in which the red blood cells(RBCs) of a fetus or neonate aredestroyed by immunoglobulin G (IgG)antibodies produced by the mother.
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Etiology
HDN is caused by :
ABO HDN Group O mother pregnant with
Group A or B baby.
Rh HDN Anti-D is the most frequent causeof severe HDN followed by Anti-c
Others Anti-K
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Pathogenesis
Fetal cells enters maternal circulation atbirth when the placenta separates fromuterus (Fetal-maternal haemorrhage)
Stimulation to produce antibody thrupregnancy or transfusion
Maternal IgG directed against fetal RBCantigens
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Pathogenesis
Ag-Ab interaction
Ab-coated RBC removed by
macrophages of spleen and liver
Anemia
Hematopoietic tissues RBC production
Immature RBC released (erythroblastosis
fetalis)
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Pathogenesis
Rate of RBC destruction decreases unless noadditional antibody entering fetal
IgG distributed EV and IV & has shelf-life of
25 days RBC hemolysed, HB released and
metabolized to indirect bilirubin
Infants unable to metabolize indirect bili asdeficient in glucuronyl transferase
Toxic level is 18mg/dL cause kernicterus
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Symptoms
Symptoms and signs in fetus:
Enlarged liver, spleen, or heart and fluidbuildup in the fetus abdomen.
Symptoms in newborn:
Anemia, Jaundice, Liver and spleen
enlargement, severe edema and Dyspnea
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Laboratory investigation
Mothers blood
ABO , RH including weak D testing
AB screening, if POS do Ab identification
If Ab identification POS, test Hb and Bili on baby
Babys blood
ABO- forward only
Rhesus including weak D if RH NEG
DAT, if POS do elution and Ab identification teston eluate
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Laboratory investigation
Qualitative test for FMH
Rosette test (qualitative test)
To detect FMH more than 30mL
Maternal suspension + Anti-D , incubated
Fetal Rh pos cells will react with Anti-D Unbound Ab washed away
Add group O, Rh pos cells
Anti-D reacts with gp O cells and fetal Rh-pos
cells in rosette pattern
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Rosette test
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Laboratory investigation
Detecting and quantifying FMH
Kleihauer-Betke (KB)-quantitative detects and measures the number of fetal
(unborn baby) cells in the mother's blood
Principle: resistance of fetal hemoglobin toacid elution
Blood film incubated at low pH, stained witheosin (appear dark), and examined
Cells containing HbF resist acid elution and
take up the stain; Cells containing adult Hb (HbA) appear as
'ghost' cells.
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Kleihauer-Betke
Hb FHb A
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Prophylaxis
Rhesus immune globulin is IgG anti-Dprepared from pooled human plasma
Two preparation 50g & 300g (IM only)
- 300g and 120g(IM or IV)
300g is protective up to 30 mL of fetal bld
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Prophylaxis
Guidelines
28th weeks of gestation 300 g
After delivery of Rh pos baby
Abortion
Miscarriage 50 g
Termination of ectopic pregnancy
Termination of pregnancy at 12th week
Amniocentesis 120 g
Other manipulations after 34
th
week
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Treatment for HDN
Intrauterine transfusion
Via intraperitoneal route or direct intravascularapproach by umbilical vein
Using group O, Rh-neg less than 7 days old CMV ab neg or leukoreduced
Gamma irradiated
Hemoglobin S negative
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Intrauterine transfusion
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Treatment for HDN
Post partum
Treatment for hyperbilirubinemia and anemia
Phototherapy accelarates bilirubin metabolism
Exchange transfusion When serum bilirubin reaches 18 to 20 mg/dL
Coated RBCs are removed and replaced by normal RBC
Reduce bilirubin
No of unblound Ab available to attach newly formedag-positive cells reduced
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Treatment for HDN
Transfusion & compatibility testing
ABO (forward) & RH (Weak D if RH neg)
Group O Rh neg is the best option or else
Ab screening using mothers or infants serum If Ab screening neg, NO crossmatching
required
If Ab screening pos, Crossmatching required
by IAT
I f t ith I H d F t li
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Infants with Immune Hydrops Fetalisdue to Rh incompatibility
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