Rh Disease

l Occurs during pregnancy when there is an incompatibility between the blood types of the mother and fetus

Blood Types

l A, B, O blood groups are specific types of proteins found on the surface of RBC’s

l Also found in the cells and other body fluids (saliva, semen, etc)

l O represents neither protein being present on RBC

l Possible groups include: A, B, AB, or O

l A, B, O groups most important for transfusions

Rh Factor

l Proteins (antigens) occurring only on surface of RBC’s

l Rh + if proteins present

l Rh – if proteins absent

l A+, A-, B+, B-, AB+, AB-, O+, O-

l Most important for pregnancy

l Inheritance is Autosomal Dominant

l 15% Caucasian population is Rh-

Nomenclature

l Correct to say Rh(D) + or –

l Rh blood system has other antigens: C, c, D, E, e

l D is by far the most common and the only preventable one

l Weak D (Du) also exists

l Also non Rhesus groups such as Kell, MNS, Duffy (Fy) and Kidd (Jk) exist

Why Does Rh Status Matters


Pathophysiology

l Rh(D) antigen expressed by 30 d GA

l Many cells pass between maternal & fetal circulation including at least 0.1 ml blood in most deliveries but generally not sufficient to activate immune response

l Rh antigen causes > response than most

l B lymphocyte clones recognizing foreign RBC antigen are formed

Pathophysiology cont…

l Initial IgM followed by IgG in 2 wks- 6 mths

l Memory B lymphocytes activate immune response in subsequent pregnancy

l IgG Ab cross placenta and attach to fetal RBC’s

l Cells then sequestered by macrophages in fetal spleen where they get hemolyzed

l Fetal anemia

Causes of RBC Transfer

l abortion/ectopic

l partial molar pregnancy

l blighted ovum

l antepartum bleeding

l special procedures (amniocentesis, cordocentesis, CVS)

l external version

l platelet transfusion

l abdominal trauma

l inadvertent transfusion Rh+ blood

l postpartum (Rh+baby)

General Screening

l ABO & Rh Ab @ 1st prenatal visit

l @ 28 weeks

l Postpartum

l Antepartum bleeding and before giving any immune globulin

l Neonatal bloods ABO, Rh, DAT

Gold Standard Test

l Indirect Coombs:

-mix Rh(D)+ cells with maternal serum

-anti-Rh(D) Ab will adhere

-RBC’s then washed & suspended in Coombs serum (antihuman globulin)

-RBC’s coated with Ab will be agglutinated

l Direct Coombs:

-mix infant’s RBC’s with Coombs serum

-maternal Ab present if cells agglutinate

+ Rh(D) Antibody Screen

l Serial antibody titres q2-4 weeks

l If titre ≥1:16 - amniocentesis or MCA dopplers and more frequent titres (q1-2 wk)

l Critical titre – sig risk hydrops

l ** amnio can be devastating in this setting

l U/S for dating and monitoring

l Correct dates needed for determining appropriate bili levels (delta OD450)

U/S Parameters

l Non Reliable Parameters:

Placental thickness

Umbilical vein diameter

Hepatic size

Splenic size

Polyhydramnios

l Visualization of walls of fetal bowel from small amounts intraabdominal fluid may be 1st sign of impending hydrops

l U/S reliable for hydrops (ascites, pleural effusions, skin edema) – Hgb <>

Amniocentesis

l Critical titre/previous affected infant

l Avoid transplacental needle passage

l Bilirubin correlates with fetal hemolysis

l ∆ optical density of amniotic fluid @ 450nm on spectral absorption curve

l Data plotted on Liley curve

Liley Curve

l Zone I – fetus very low risk of severe fetal anemia

l Zone II – mild to moderate fetal hemolysis

l Zone III – severe fetal anemia with high probability of fetal death 7-10 days

l Liley good after 27 weeks

l 98% sensitive for detecting anemia in upper zone 2/ zone 3

Middle Cerebral Artery Dopplers

l Measures peak velocity of blood flow

l Anemic fetus preserves O2 delivery to brain by increasing flow

l Sensitivity of detecting severe anemia when MCA >1.5 MoM approaches 100%

l Not reliable > 35 weeks GA

Fetus at Risk

l Fetal anemia diagnosed by:

¡ amniocentesis

¡ cordocentesis

¡ ultrasound

hydrops

middle cerebral artery Doppler

l Treatment:

¡ intravascular fetal transfusion

¡ preterm birth

Infant at Risk

l Diagnosis:

¡ history of HDN antibodies?

¡ early jaundice <>

¡ cord DAT (“Coomb’s”) positive (due to HDN or ABO antibodies)

l Treatment:

¡ Phototherapy

¡ Exchange or Direct blood transfusion

Prevention

l RhoGAM (120mcg or 300mcg)

l Anti-D immune globulin

l Previously 16% Rh(D)- women became alloimmunized after 2 pregnancies, 2% with routine PP dose, and 0.1% with added dose @ 28 wks

Kleihauer-Betke Test

l % fetal RBC in maternal circulation

l Fetal erythrocytes contain Hbg F which is more resistant to acid elution than HbgA so after exposure to acid, only fetal cells remain & can be identified with stain

l 1/1000 deliveries result in fetal hemorrhage > 30ml

l Risk factors only identify 50%

Kleihauer Calculations

l Fetal red cells = MBV X maternal Hct X % fetal cells in KB

newborn Hct

MBV – maternal blood volume (usually 5000ml)

Fetal cells X 2 = whole blood