HEMOLYTIC DISEASE OF THE NEWBORN (HDN)

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

Content
Definition of HDN Classification Etiology and pathogenesis of HDN Laboratory investigation

Neonatal test Maternal blood test

Exchange transfusion

Definition

Also known as erythroblastosis fetalis (presence of nucleated RBCs), or hydrops fetalis (edema)

Is a condition in which the red blood cells (RBCs) of a fetus or neonate are destroyed by immunoglobulin G (IgG) antibodies produced by the mother.

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 cause of severe HDN followed by Anti-c

Others Anti-K

Pathogenesis
Fetal cells enters maternal circulation at birth when the placenta separates from uterus (Fetal-maternal haemorrhage) Stimulation to produce antibody thru pregnancy or transfusion Maternal IgG directed against fetal RBC antigens

Pathogenesis
Ag-Ab interaction

Ab-coated RBC removed by macrophages of spleen and liver

Anemia

Hematopoietic tissues

RBC production

Immature RBC released (erythroblastosis fetalis)

Pathogenesis
Rate of RBC destruction decreases unless no additional 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 as deficient in glucuronyl transferase Toxic level is 18mg/dL cause kernicterus

Symptoms

Symptoms and signs in fetus:

Enlarged liver, spleen, or heart and fluid buildup in the fetus abdomen.

Symptoms in newborn:

Anemia, Jaundice, Liver and spleen enlargement, severe edema and Dyspnea

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 test on eluate

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

Rosette test

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 to acid elution Blood film incubated at low pH, stained with eosin (appear dark), and examined Cells containing HbF resist acid elution and take up the stain; Cells containing adult Hb (HbA) appear as 'ghost' cells.

Kleihauer-Betke

Hb A

Hb F

Prophylaxis

Rhesus immune globulin is IgG anti-D prepared 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

Prophylaxis

Guidelines

28th weeks of gestation After delivery of Rh pos baby Abortion Miscarriage Termination of ectopic pregnancy

300 g

50 g

Termination of pregnancy at 12th week Amniocentesis Other manipulations after 34th week

120 g

Treatment for HDN

Intrauterine transfusion

Via intraperitoneal route or direct intravascular approach by umbilical vein Using group O, Rh-neg less than 7 days old CMV ab neg or leukoreduced Gamma irradiated Hemoglobin S negative

Intrauterine transfusion

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 formed ag-positive cells reduced

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

Infants with Immune Hydrops Fetalis due to Rh incompatibility

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