Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Immune hydrops (erythroblastosis fetalis, hemolytic disease of the newborn)
Introduction

Hemolytic disease of the newborn is caused by specific maternal antibodies directed against red cell antigens of the fetus. These are largely Rh (D) antigens.

Etiology, pathogenesis
  • Pre-requisite of Rh isoimmunization: Rh- mother and Rh+ fetus.
  • The mother is sensitized to Rh antigen by Rh+ fetal erythrocytes that reach the maternal circulation. The mother is thus stimulated to production of anti-Rh antibodies. Maternal IgG antibodies cross the placenta, attach to the red blood cells and lead to hemolytic anemia in the fetus. Compensatory mechanisms used by the fetus include extensive activation of the hematopoeitic tissue and increased cardiac output. Congestive heart failure and generalized edema are marks of decompensation. The sensitization occurs most often during the first childbirth, so the second and any following pregnancy are at risk of hemolytic disease. The immunization also develops following invasive examination durign pregnancy and abortion but the volume of transplacental bleed is lower than during childbirth. There is a very effective preventive program of Rh-isoimmunization. Intravenous drug abuse in young women has been reported as the most common cause of Rh isoimmunization today.

Rare causes of erythroblastosis fetalis: Isoimmunisation against other antigens of the Rh system (C, E) or antigens Kell, Lewis occurs rarely. The clinical signs are the same as for the original anti-D disease.

ABO incompatibility: Pre-requisite: Mother of group O and infant of group A or B. Hemolytic disease of the newborn in cases of ABO incompatiblity is usually mild. It presents as fetal anemia in the third trimestr of pregnancy and moderate jaundice after delivery. Hydrops fetalis is an exceptional complication.

Clinical signs
  • Clinical stages of the hemolytic disease of the newborn:
    • anemia neonatorum
    • icterus neonatorum
    • hydrops fetus universalis: the most severe stage of the hemolytic disease; a hydropic fetus is stillborn or dies soon after birth
  • Prevention: Administration of the anti-Rh (D) immunoglobulin to Rh- women with Rh+ newborn within 72 hours of delivery, also to Rh- women following 2nd trimestr abortion, chorionic villi sampling, amniocentesis and cordocentesis
Macroscopic appearance
  • generalized tissue edema
  • hepatosplenomegaly
  • cardiomegaly
  • the placenta is enlarged, pale and very friable
Pictures
Histology

Increased extramedullary hematopoiesis. Hepatic sinusoids are crowded by immature nuclated red cells precursors.

Placenta: Villous immaturity, abundance of Hofbauer cells, persistent cytotrophoblastic layer, deacreased vascularity, nucleated red blood cells within the vessels.

Spleen with extramedullar hemopoiesis (72722)

Pictures

Fetal erythroblastosis, spleen with extramedullar hemopoiesis, placenta with nucleated erythrocytes: Fetal erythroblastosis, spleen, HE 100x (72722) Fetal erythroblastosis, placenta, HE 100x (72739)