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Hemolytic Disease of the Newborn

*Morbus haemolyticus neonatorum*

For medical students2 min readUpdated 2026-10-10

A pathological condition arising from maternal-fetal blood group incompatibility. It is characterized by enhanced destruction of the infant's erythrocytes (hemolysis), leading to anemia and jaundice, and in severe cases, intrauterine fetal demise or profound neurological impairment.

LiverMarked extramedullary hematopoiesis in the fetus and premature infant
EnzymesInsufficient glucuronyl transferase activity exacerbates jaundice
HemoglobinBreakdown of fetal hemoglobin contributes to hyperbilirubinemia

Causes and Mechanisms of Jaundice

The pathogenesis of jaundice is viewed in the context of internal organ immaturity, primarily the liver. In premature infants, the liver is relatively large, but its enzyme systems do not function at full capacity.

The main factor determining a prolonged and pronounced course of jaundice is the insufficient activity of the enzyme glucuronyl transferase. Normally, this enzyme is responsible for the conjugation and excretion of bilirubin, which is formed as a result of the massive breakdown of fetal hemoglobin after birth.

Morphological Features of the Liver

Histological examination of the liver of a fetus or premature infant (with hematoxylin and eosin staining) reveals a characteristic picture.

The main morphological feature is the presence of pronounced extramedullary hematopoiesis. Multiple foci of blood cell formation are visualized in the liver tissue, indicating stressed functioning of the fetal hematopoietic system.

Features of Other Organs in Immature Fetuses

Because hemolytic disease is frequently associated with prematurity or immaturity of fetal systems, it is important to note the morphofunctional characteristics of other organs:

Frequently asked questions

What are the clinico-anatomical forms of hemolytic disease of the newborn?

Three main clinical forms of hemolytic disease of the newborn are distinguished based on their frequency of occurrence:

  • Icteric form — occurs most frequently (88% of cases).
  • Anemic form — observed in 10% of patients.
  • Hydrops fetalis — the rarest form, diagnosed in 2% of cases.
What microscopic changes are detected in the liver in hemolytic disease of the newborn?

Microscopic examination of the liver reveals abnormally high erythropoietic activity with the formation of prominent foci of extramedullary hematopoiesis. In addition, as a result of intravascular hemolysis, generalized hemosiderosis develops, characterized by the formation and accumulation of hemosiderin pigment in macrophages and Kupffer cells (hepatic stellate cells).

Which brain structures are affected in the development of kernicterus?

During the development of kernicterus, unconjugated bilirubin causes toxic damage primarily to subcortical nuclei and other specific areas of the brain. The affected structures include:

  • Basal ganglia — specifically the globus pallidus.
  • Thalamus and subthalamic nuclei.
  • Cerebellum and hippocampi.
  • Spinal cord and cranial nerves (VIII, VII, and III).
  • Gray matter of the cerebral cortex — affected less frequently.

Macroscopically, these areas acquire a bright yellow coloration, and the brain itself becomes edematous.

What morphological changes occur in the spleen in hemolytic disease of the newborn?

The main macroscopic change in the spleen in hemolytic disease of the newborn is its pronounced enlargement — splenomegaly (as part of hepatosplenomegaly). Microscopically, the organ shows:

  • Extramedullary erythropoiesis — the formation of foci of extramedullary hematopoiesis as a compensatory reaction to hemolysis and anemia.
  • Hemosiderosis — the formation and accumulation of hemosiderin pigment in splenic macrophages due to intravascular erythrocyte destruction.
Which type of maternal antibody crosses the placenta and causes hemolysis of fetal erythrocytes?

Hemolysis of fetal erythrocytes is caused by maternal IgG antibodies. This is the only class of immunoglobulins capable of crossing the placental barrier. In the presence of maternal-fetal blood group incompatibilities (such as Rh incompatibility or ABO incompatibility), these antibodies enter the fetal circulation, bind to fetal red blood cells, and cause their destruction, leading to hemolytic disease of the newborn.

Why is jaundice more severe in premature infants?

This is due to the marked immaturity of hepatic enzyme systems, specifically the insufficient activity of glucuronyl transferase.

What is extramedullary hematopoiesis?

It is the process of blood cell formation outside the bone marrow, such as in the liver, which is a characteristic histological feature of immature fetuses.

What structures are characteristic of the fetal adrenal glands?

The fetal adrenal glands are characterized by the presence of a prominent fetal cortex, whose cells are arranged in radial cords and undergo regression after birth.

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