CLS 306 (BB) - Lecture 11: Hemolytic Disease of the Fetus and Newborn

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44 Terms

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Erythroblastosis Fetalis

Disease when maternal antibodies destroy fetal RBCs, either in-utero or ex-utero (postpartum); fetal RBCs have antigens foreign to the mother that results in teh stimulation of maternal antibody production

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Hydrops Fetalis

The effect of erythroblastosis fetalis on the fetus that includes general anemia, possible edema, possible cardiac failure, and up to fetal death

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  • anti-E

  • anti-c

  • anti-K

  • anti-Jk

  • anti-Fy

Immune allo (“other”) Abs that cause HDFN are usually due to which antibodies? (5)

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Anti-K

Second most common form of severe HDFN; disease ranges from mild to severe; over half of the maternal cases that develop this antibody are caused by previous multiple blood transfusions

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  • Fetal severe anemia

  • Fetal heart failure

  • Fetal death

Dangers of HDFN in-utero (pre-partum)

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Unconjugated (indirect) bilirubin

As a result of the destroyed RBCs in HDFN, (——) is released into the fetal body

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Unconjugated bilirubin is sequestered out via the mother’s liver

Why is unconjugated bilirubin not as harmful in in-utero HDFN, as opposed to post-partum HDFN?

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  • ABO, Rh, ABS tests performed on first obstetrical visit

    • ABS (-) → repeat 24 weeks

    • ABS (+) → AB ID, if IgG → AB titer

Maternal prenatal care/testing (3)

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>1:32

Ab titer significant for HDFN maternal prenatal testing

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Last previous titer sample

(Maternal prenatal testing) When performing the current titer, mist run (——) in parallel with current titer sample

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amniocentesis at 18-20 weeks gestation

Based on maternal Ab titer results, if HDFN is suspected then a (——) is performed

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Amniotic fluid bilirubin

(——) is a direct indicator of the severity of HDFN

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ΔOD at 450 nm

What is observed when testing for bilirubin (risk of HDFN) in amniotic fluid through a spectrophotometer?

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  • Liley’s graph

  • Queenan curve

What is used to help interpret the data from spectrophotometric analysis of bilirubin in amniotic fluid (2 charts)

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  • Fetal hydrops detected on ultrasound

  • Fetal Hb <10 g/dL (severe anemia)

  • ΔOD 450 is high on Liley’s graph or Queenan curve (hyperbilirubinemia)

Intrauterine transfusion is necessary when one or more of the collowing conditions are met (3)

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  • <5 days old

  • CPDA-1 or AS-3 additive only

  • Ag negative to maternal allo Ab

  • O neg

  • CMV neg

  • Hb S neg

  • Irradiated

Intrauterine blood requirements (7)

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  • Anemia

  • Hepatomegaly

  • Splenomegaly

  • Jaundice

  • Kernicterus

Maternal Ab(s) continue to destroy fetal RBCS postpartum during HDFN. What are the signs and symptoms that occur? (5)

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  • Phototherapy (at 460-490 nm)

  • Exchange transfusions

Choices of treatment for neonatal hyperbilirybinemia are… (2)

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  • Lipophilic

  • Toxic to the brain

Phototherapy is used to change unconjugated bilirubin to an isomer which is less (——) and less (——)

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ABO HDFN

Most common form of HDFN; can occur at the 1st delivery and subsequent pregnancies; no prior exposure needed

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O type mother with Anti-A,B IgG, delivering A neonate (B less common)

How is ABO HDFN commonly caused?

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Less

ABO HDFN is usually (more/less/equal to) Rh HDFN

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It is not predictive of HDFN (not significant, no titers done)

What is the significance of maternal prenatal testing in predicting ABO HDFN?

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  • Repeat ABO/Rh and ABS (mother)

  • Determine ABO/Rh, including weak D (baby)

  • Perform DAT (baby)

    • if IgG DAT positive → elution procedure against A1 and B cells

Postpartum lab testing (cord blood workup) for ABO HDFN (cord blood work-up) (3)

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heat/chemicals dissociate Ab from baby RBCs, and supernatant used for Ab ID (Ab found is of maternal origin)

What is the elution procedure during postpartum testing of ABO HDFN?

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  • Not preventable

  • Commonly treatable by phototherapy only

  • ≥20 mg/dL serum bilirubin (rare) requires exchange transfusion

ABO HDFN prevention and treatment (3)

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  • anti-D

  • C, c, E, e

Most common cause of Rh HDFN is (——), but Abs against (—4—) antigens are known to cause it as well

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Rh HDFN

Most severe form of HDFN; severe anemia may occur resulting in severe hyperbilirubinemia and jaundice; kernicterus could result

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  • 2nd or subsequent pregnancies

    • Must consider miscarriage, abortion, prenatal fetal/maternal bleed

When does Rh HDFN typically manifest

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  • Anti-C = rare

  • Anti-c = mild to severe

  • Anti-E = mild

  • Anti-e = rare

    • Ab combinations can be severe

Severity of Rh HDFN due to anti- C, c, E, and e

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  • ABO, Rh (Du), ABS

  • if ABS (+)

    • AB ID and titer (repeat titer every 4 weeks)

    • Ag status of father (optional)

    • Ig subclass of maternal Ab

Rh HDFN prenatal testing (4)

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  • Middle Cerebral Artery-Peak Systolic Velocity (MCA-PSV)

  • Amniocentesis

If anti-d (or anoy other Rh AB) titer reaches >1:32, consider further testing… (2)

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Middle Cerebral Artery-Peak Systolic Velocity (MCA-PSV)

(Rh HDFN testing) color doppler/ultrasound transducer that is a reliable predictor of fetal anemia; favored over amniocentesis because it is noninvasive and gives tehe same information

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  • Repeat ABO/Rh and ABS

  • If ABS positive, must do Ab ID

    • weak anti-D may be due to Rh Immune Globulin shot (Rhogam) = “passive” anti-D

  • Enumerate # fetal cells in mother’s blood

Postpartum testing for Rh HDFN in mother (3)

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  • Determine ABO/Rh (including weak D)

  • Perform DAT

    • DAT + → elution test against Ab ID panel cells

Postpartum lab testing (cord blood work-up) on the baby for Rh HDFN (2)

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Iatrogenic anemia

Some hospitals may not perform an elution test on baby RBCs for allo Ab ID because it can cause…

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Fetal Hgb Screen Test/Rosette Test

Qualitative test that demonstrates small number of D positive cells in mother’s D negative cell suspension

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Kleihauer-Betke test

Quantitative test where the blood sample from the mother is treated with acid and stained to show any present fetal red cells (resistant to acid); determine # of fetal cells in first 2,000 maternal cells counted

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Volume fetal cells = # fetal cells * maternal blood volume / 2,000

Equation used for the Kleihauer-Betke test

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  • Hb < 10 g/dL

  • Bilirubin ≥20 mg/dL

Exchange transfusion can occur when either conditions exist… (2)

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  • Type AB

  • Rh can be disregarded

  • No irradiation

Requirements of plasma transfusion for neonate (3)

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  • Removes 50% bilirubin

  • Removes 80-90% infant’s sensitized RBCs

  • Removes 80-90% maternal incompatible Ab

  • Replacement of incompatible RBC with compatible RBC

Beneficial effects of a 2-volume exchange transfusion in an HDFN neonate (4)

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  • Avoid clinically significant hemodynamic shifts

  • Avoid any metabolic abnormalities

The neonatal exchange transfusion process is very slow in order to…

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  • Donor blood → venous catheter

  • Arterial catheter → drainage/waste container

Direction of blood entering/exiting the baby during exchange transfusion