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Updated: Jul 29 2022

Rh Disease

  • Snapshot
    • A 30-year-old G2P1 woman presents to the emergency room with contractions at 37 weeks. She previously had a baby boy via spontaneous vaginal delivery without complications. She had this delivery done in a foreign country, and obstetric records are not available. Her prenatal care has also been limited. On fetal ultrasound, it is noted that the fetus has ascites and soft tissue edema. She is immediately prepped for delivery.
  • 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 a Rh-negative mother is pregnant with a 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
    • Rosette test
      • to detect fetal-maternal hemorrhage
    • Kleihauer-Betke test
      • if Rosette test is positive, can conduct this 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
          • only if mother is Rh-negative and has not been sensitized previously, given at 28th week of pregnancy, if possible, and within 72 hours after birth 
          • external cephalic version
          • amniocentsis
          • ectopic pregnancy
      • 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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