Agranulocytosis in children

Agranulocytosis in children is represented by several types of the disease:

• Kostmann syndrome or childhood genetically determined agranulocytosis.
• Childhood agranulocytosis with cyclic neutropenia.
• Agranulocytosis in newborns with isoimmune conflict.

Let's take a closer look at each type of disease.

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Kostmann syndrome

A very serious disease that is transmitted by an autosomal recessive type of inheritance. This means that both parents of a sick child are healthy people, but at the same time, they find themselves as carriers of a pathological gene.

Symptoms of the disease in children are expressed in the following manifestations:

• In newborns, purulent infections occur, among which one can observe the appearance of recurrent skin lesions along with the formation of pustules and inflammatory infiltrates.
• Also characteristic of the disease are symptoms of ulcerative stomatitis, infectious periodontitis and periodontosis, the appearance of hypertrophy and bleeding gums.
• Among the pathologies of the respiratory organs, the appearance of otitis, mastoiditis, inflammatory processes that occur in the mucous membrane of the nose are characteristic. It is also possible to develop protracted forms of pneumonia, which are prone to the formation of abscesses.
• In this case, children experience an increase in body temperature, accompanied by a generalized enlargement of the lymph nodes. In some cases, a certain degree of splenomegaly is observed - an enlargement of the spleen.
• Severe forms of the disease provoke the development of septicemia, in which abscesses form in the liver.
• The white blood cell count in the blood begins to change. Typical cases are characterized by the presence of an extreme degree of neutropenia, in which neutrophils are not detected at all. Eosinophilia and monocytosis are also noted against the background of a normal level of lymphocytes in the blood.

Currently, scientists have developed a drug called granulocyte colony-stimulating factor (G-CSF), which is used in the treatment of this disease.

Childhood agranulocytosis associated with cyclic neutropenia

Cyclic neutropenia is a disease that is hereditary. Its occurrence is provoked by an autosomal dominant type of inheritance of traits: the disease appears in a child only if at least one of the parents has a similar pathology.

The clinical picture of the disease looks like this: there is a rhythmic fluctuation in the number of neutrophils in the blood plasma - from normal to a deep degree of agranulocytosis, when the presence of these particles is not determined at all.

Symptoms of cyclic agranulocytosis are periodically recurring fever, ulcerative lesions of the oral cavity and larynx, enlargement of regional lymph nodes and obvious symptoms of intoxication. When the fever subsides, the child's condition returns to normal. But some small patients get complications in the form of otitis, abscessing pneumonia, etc. instead of high temperature.

The most important sign of cyclic neutropenia is periodic decreases in the number of granulocytes in the blood plasma. In its most extreme stage, agranulocytosis appears, which lasts for three to four days. After which neutrophils begin to appear in the blood again, but in small quantities. At the same time, an increase in the number of lymphocytes is possible. After this peak phase, laboratory tests can reveal transient monocytosis and eosinophilia. And then, until the onset of the next cycle, all blood parameters return to normal. In some patients, agranulocytosis is accompanied by a decrease in the level of red blood cells and platelets.

Therapy for this disease in children consists primarily of preventing complications from infections that have entered the body. In addition, the drug granulocyte colony-stimulating factor (G-CSF), which reduces the degree of granulocytopenia, has proven itself well. Unfortunately, this medication cannot prevent the occurrence of cyclic changes in the blood composition of a child.

Agranulocytosis in newborns with isoimmune conflict

With isoimmune neutropenia, there is pronounced granulocytopenia, which can reach the stage of agranulocytosis. This disease is characterized by the same symptoms as hemolytic anemia in newborns with Rh-conflict, only in this case the problem occurs with granulocytes.

The mechanism of this pathology is the formation in the mother's body of antibodies to children's granulocytes, which have the same antigen composition as the baby's father. The antibodies are directed to the granulocyte precursor cells produced by the red bone marrow.

The peculiarities of this type of agranulocytosis are manifested in the transitory nature of the disease. In this case, maternal antibodies are washed out of the child's body by fluids at a fairly high speed, which stimulates spontaneous normalization of the number of granulocytes in the patient's blood plasma. Therefore, the child recovers ten or twelve days after the onset of the disease. At this time, it is important to use antibiotics to prevent infectious complications.

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