RH INCOMPATIBILITY

(ERYTHROBLASTOSIS FETALIS)
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
It is an immunologic disorder
characterized by excessive haemolysis
of fetal RBCs by antibodies that pass
through the placenta from maternal
blood. Erythroblastosis foetalis (also
called haemolytic disease of the newborn),
caused by Rh alloimmunization, is an
important cause of infant morbidity and
mortality.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
THE RHESUS FACTOR:
The Rhesus (Rh) factor is a complex antigen
that is present on the surface of RBCs. It
consists of 3 pairs of genes Cc, Dd, Ee, the most
important of which is the D. The D gene is
dominant therefore an Rh-positive individual
may be homozygous (DD) or heterozygous (Dd)
and an Rh-negative individual has a (dd)
genotype. The C and E antigens may also lead
to incompatibility. But they are quite rare.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
AETIOLOGY:
Rh-negative females develop anti-Rh
antibodies if they were subjected to:
Blood transfusion with an Rh-positive
blood. The immune system responds by
the production of antibodies against the D
antigen.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Married to Rh-positive male and get
pregnant in Rh-positive baby. At time of
delivery or (also disturbance of ectopic
pregnancy, antepartum haemorrhage,
amniocentesis, or external cephalic
version) foeto-maternal haemorrhage
occurs (passage of fetal RBCs to maternal
circulation). These fetal Rh-positive RBCs
stimulate the immune system to produce
antibodies against the Rh-positive antigen.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
INCIDENCE:
85% of the Caucasian population is Rh-
positive while 15% is Rh-negative.
However, the incidence of erythroblastosis
foetalis is less than 1% due to:
An Rh-negative female may get married to
an Rh-negative male.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
An Rh-negative female may get married to
an Rh-positive heterozygous (Dd) male.
The baby has a 50% chance of being Rh-
negative.
ABO incompatibility between the mother
and her foetus results in the haemolysis of
transfused fetal cells into the maternal
circulation before they can induce Rh
antibody response.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Immunologic variation in the response to
Rh-positive antigen. Some Rh-negative
women are non-responders and others are
to poor responders.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
The first baby is usually unaffected because on
the first exposure to Rh antigen, during the first
pregnancy, the immune system responds by
producing IgM antibodies (first immune
response) that have a large molecular weight
so can not cross the placenta. While on the
second exposure, the immune system
responds by producing IgG antibodies (second
immune response) that have a small molecular
weight thus cross the placenta affecting the
second baby.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
CLINICAL TYPES:
In all types, haemolysis of fetal Rh-positive RBCs occurs
due to the passage of anti Rh antibodies from maternal
to fetal circulation in a previously sensitised pregnant
woman.
I. Congenital haemolytic anaemia: (Mildest form) :
Haemolysis results in fetal anaemia that develops 2
weeks after birth.
Due to faster rate of erythropoiesis, erythroblasts
(immature nucleated RBCs) are produced, hence, the
name erythroblastosis foetalis.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
II. Icterus gravis neonatorum:
(Commonest and moderate form) :

The baby is delivered anaemic but never

jaundiced at birth as the placenta carries on the
function of conjugating fetal bilirubin.
Hepatosplenomegaly is usually present due to
faster erythropoiesis.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Jaundice develops within 48 hours after birth
due to the inability of the fetal liver to conjugate
the high amount of bilirubin produced by the
haemolysed RBCs.
kernicterus develops when fetal bilirubin level
exceeds 20 mg%. Bilirubin crosses the blood
brain barrier and becomes deposited in the
basal ganglia of the brain stem, a serious
condition that may lead to neonatal death or
mental retardation.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
III. Hydrops foetalis: (Severest form)
Intrauterine fetal death usually occurs due
to severe haemolytic anaemia that leads
to heart failure. If the baby was born alive,
it dies within few hours.
The foetus shows generalized oedema,
pleural effusion and ascites due to heart
failure.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Hepatosplenomegaly.
The placenta is large and oedematous.
If ultrasound is done during pregnancy the
foetus shows the "Buddha" attitude due to
flexion of the thighs and abdominal
distension and shows a halo around the
skull due to scalp oedema.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
DIAGNOSIS DURING PREGNANCY:
Rh group should be checked in all
pregnant women at the first antenatal visit
and if Rh-negative:
Ask about previous blood transfusion, fetal
anaemia, neonatal jaundice or IUFD.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Determine the Rh group of the husband and if
positive proceed for:
Indirect coomb's test: to detect the presence
and the titre of anti-Rh antibodies (Anti-D IgG). If
the titre =1/16 amniocentesis is indicated. If
<1/16 repeat the test every 4 weeks.
Amniocentesis: is done, if antibody titre is
>1/16, to determine the amount of bilirubin in the
amniotic fluid that reflects the degree of
haemolysis in fetal blood.
U.S: May show fetal hepatosplenomegaly,
oedema, or Buddha attitude.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
DIAGNOSIS AFTER LABOUR:
Cord Blood is obtained for Rh grouping and if
positive:
Assess haemoglobin level to detect fetal
anaemia (normal 18 gm%),
Assess serum bilirubin to detect jaundice
(normal = 2 mg%).
Direct Coombs test: detects the antibodies
absorbed to the RBCs (sensitisation).
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
PROPHYLAXIS AGAINST
ERYTHROBLASTOSIS FOETALIS:
Rh-negative females should never receive
Rh-positive blood transfusion.
Anti-D immunoglobulins should be given to
all Rh-negative non-sensitised (negative
indirect coomb's test) females married to
Rh-positive males in the following
conditions:
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
After delivery of an Rh-positive baby to destroy
fetal RBCs before they initiate maternal immune
response (300 mcg Anti-D I.M. within 72 hours
of delivery).
At time of abortion, disturbance of ectopic
pregnancy, antepartum haemorrhage,
amniocentesis, or version (50-100 mcg Anti-D
I.M.).
Recently, 300 mcg of Anti-D immunoglobulins
I.M. are given at 28 weeks of pregnancy is
recommended to prevent sensitisation during
pregnancy.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
TREATMENT DURING PREGNANCY:
Intrauterine blood transfusion:
This is indicated if the foetus is severely
affected before 34 weeks gestation. Rh-
negative group O blood is injected into the
peritoneal cavity of the foetus or into the
umbilical vein. It is done under
ultrasonographic guidance and local
anaesthesia.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
Termination of pregnancy:
It is indicated if the foetus is severely
affected after 34 weeks gestation.
Induction of labour or caesarean section is
done when lung maturity is demonstrated
by L/S ratio.
 RH INCOMPATIBILITY
(ERYTHROBLASTOSIS FETALIS)
NEONATAL MANAGEMENT:
Exchange transfusion:
10-20 ml of blood are withdrawn from the
umbilical vein and replaced with the same
amount of Rh-negative group O blood and
repeated till 80-90% of the blood is replaced.
The aim is to remove the antibodies, remove the
antigen (replace the Rh-positive cells with Rh-
negative ones), correct anaemia, and correct
jaundice (remove bilirubin).
 Destruksi sel-sel darah merah
anemia, hipertrofi hepar penekanan
sel-sel normal hepar destruksi dan
penurunan produksi protein hidrops
fetalis
Patogenesis utama :
Perubahan aliran limfe
Peningkatan tekanan hidrostatik
intravaskuler
Penurunan tekanan osmotik intravaskuler
 Aliran cairan pada tingkat kapiler
berhubungan langsung dengan
tekanan hidrostatik intravaskuler dan
tekanan osmotik dalam cairan intertitiel
Berbanding terbalik dengan tekanan
hidrostatik intertitial dan tekanan
osmotik koloid plasma
Keseimbangan ruang intravaskuler
janin dan ibu melalui plasenta
 Hiperplasia plasenta terjadi akibat
upaya untuk meningkatkan transfer
oksigen
Malformasi jantung kongenital dan
massa di rongga toraks gangguan
hemodinamik akibat obstruksi aliran
darah balik vena dan penurunan
perfusi jaringan perifer
 Hidrotoraks janin dan
anomali toraks
Dapat berdiri sendiri atau berhubungan
dengan hidrops
Meningkatkan tekanan intratorak dan
mengganggu aliran balik vena
Penekanan mediastinum gangguan
menelan polihidramnion