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Updated: Aug 12 2021

Rh Hemolytic Disease of the Newborn

  • Snapshot
    • A 32-year-old G1P0 woman presents to the emergency room with contractions. She was found to be Rh-negative and her husband’s Rh status is unknown. Fetal ultrasound shows no signs of edema or ascites. After the Kleihauer-Betke test, she is given the appropriate dose of Rh IgG.
  • Introduction
    • Overview
      • Rh hemolytic disease of the newborn is caused by Rh incompatibility
      • maternal anti-Rh IgG antibodies cross the placenta and destroy fetal Rh-positive red blood cells
    • Epidemiology
      • incidence
        • 15% of the population is Rh-negative
      • risk factors
        • history of prior blood transfusion
        • previous pregnancy
        • mother is Rh-negative, and father is Rh-positive or unknown
        • prior administration of Rh IgG (RhoGam)
        • history of invasive obstetric procedures
    • Pathogenesis
      • mechanism
        • Rh factor is a red blood cell antigen
        • when an Rh-negative mother is pregnant with an Rh-positive fetus, the mother is exposed to Rh-positive red blood cells and leads to maternal antibody production (IgG) against the foreign Rh antigen
        • as IgG can cross the placenta, subsequent pregnancy with Rh-positive fetus will result in fetal alloimmune-induced hemolytic anemia
        • development of antibody depends on volume of transplacental crossover of red blood cells, concurrent presence of ABO incompatibility, and extent of maternal immune response
        • breakdown of red blood cells causes elevation of bilirubin
  • Presentation
    • Symptoms
      • hemolytic anemia
    • Physical exam
      • inspection
        • jaundice
        • pallor
  • Imaging
    • Fetal ultrasound
      • indication
        • suspected Rh incompatibility
      • findings
        • fetal ascites and edema
  • Studies
    • Serum labs
      • hyperbilirubinemia
      • low hematocrit
      • elevated reticulocyte count
      • positive direct Coombs test in fetus; positive indirect Coombs test in the mother
    • Rosette test
      • initial test to test for fetal-maternal hemorrhage
    • Kleihauer-Betke test
      • measures fetal red blood cells in utero in maternal circulation to determine dose of RhoGAM
  • Differential
    • ABO incompatibility
      • key distinguishing factor
        • typically less severe
  • Treatment
    • Medical
      • maternal anti-D immune globulin (Rh IgG or RhoGAM) administration
        • indication
          • if mother is Rh-negative and has not been sensitized previously, given at 28th week of pregnancy, if possible, and 72 hours after birth
          • external cephalic version
          • amniocentsis
          • ectopic pregnancy
          • any suspected / known exposure of Rh-positive blood
      • exchange transfusion
        • indications
          • erythroblastosis fetalis
          • hydrops fetalis
          • kernictus
  • Complications
    • Kernicterus secondary to hyperbilirubinemia
      • loss of Moro reflex
      • posturing
      • poor feeding
      • seizures
    • Erythroblastosis fetalis
      • most severely, can manifest as hydrops fetalis with high output cardiac failure, edema, and death
      • indication for immediate delivery and exchange transfusion
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