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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