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Erythroblastosis Fetalis and Hydrops Fetalis

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Pathology of the Human Placenta

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Abstract

Erythroblastosis fetalis, or hemolytic disease of the newborn, is a condition caused by specific antibodies of the mother, directed against red cell antigens of the fetus. These are largely Rh-(D) antigens, but rare cases of sensitization against other antigens (e.g., Kell), and of ABO incompatibility with fetal hemolytic disease have been described. Leventhal and Wolf (1956) have presented Kell isoimmunization as a cause of fatal erythroblastosis (EF). This form was also found in the well-illustrated case of Ivemark et al. (1959). Anti-K antibodies usually arise as a result of transfusion, and the fetal disease is usually mild. Of 194 pregnancies complicated by this antibody constellation, only 16 affected babies were identified, of which 3 were severely affected by hemolytic disease (Leggat et al., 1991). The difficulty in this situation is the identification of the pregnancies at risk, an aspect discussed in some detail in an Editorial (1991). Anti-K hemolytic disease does not differ histopathologically from anti-D-caused erythroblastosis. There may, however, be a difference in the response to this antibody. Vaughan et al. (1998) showed that they specifically inhibit growth of K-positive erythroid precursors and may thus lead to severe fetal anemia. Relatively few cases of typical, severe EF have been described as being due to ABO incompatibility. These cases were summarized by Freda and Carter (1962) and a fatal case is delineated in the paper by Miller and Petrie (1963), but usually the hemolytic disease of ABO incompatibility is mild. The pathological findings of infant and placenta are the same as those in EF caused by Rh incompatibility; in the case described by Miller and Petrie, the placenta weighed 900g and had typical features of erythroblastosis. Other types of hemolysis occur that also produce similar pathological features of infant and placenta. Thus, hemolysis in fetal blood may rarely result because of glucose-6phosphate dehydrogenase (G-6-PD) deficiency, virus infection, or for other uncommon reasons. These causes of fetal hemolysis must be differentiated from the classical erythroblastosis.

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Authors and Affiliations

  1. University Medical Center, University of California, San Diego, 200 West Arbor Drive, 92103-8321, San Diego, California, USA

    Kurt Benirschke MD (Professor Emeritus of Pathology and Reproductive Medicine, Emeritus)

  2. Institut für Anatomie der Medizinischen Fakultät, Rheinisch-Westfälische Technische Hochschule Aachen, Wendlingweg 2, 52057, Aachen, Germany

    Professor Dr med Peter Kaufmann

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Benirschke, K., Kaufmann, P. (2000). Erythroblastosis Fetalis and Hydrops Fetalis. In: Pathology of the Human Placenta. Springer, New York, NY. https://doi.org/10.1007/978-1-4757-4199-5_16

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  • DOI: https://doi.org/10.1007/978-1-4757-4199-5_16

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