Agranulocytosis in children

Agranulocytosis in children is represented by several types of disease:

• Kostmann's syndrome or child's genetically determined agranulocytosis.
• Children's agranulocytosis on a background of cyclic neutropenia.
• Agranulocytosis in newborns with isoimmune conflict.

Let us consider in detail each type of disease.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10],

Kostmann's syndrome

A very serious disease that is transmitted through 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:

• Newborns have purulent infections, among which one can observe the appearance of recurrent skin lesions along with the formation of pustules and inflammatory infiltrates.
• Symptoms of ulcerative stomatitis, infectious periodontitis and periodontitis, the appearance of hypertrophy and bleeding gums are also characteristic for the disease.
• Among pathologies of the respiratory organs, otitis, mastoiditis, inflammatory processes that occur in the nasal mucosa are characteristic. It is also possible the emergence of protracted forms of pneumonia, which are prone to the formation of abscesses.
• In children, the body temperature rises, accompanied by a generalized enlargement of the lymph nodes. In some cases, there is a certain degree of splenomegaly - an increase in spleen.
• Heavy forms of the disease provoke the development of septicemia, in which abscesses are formed in the liver.
• The formula of leukocytes in the blood begins to change. Typical cases are characterized by an extreme degree of neutropenia, in which neutrophils are not determined at all. Also, the occurrence of eosinophilia and monocytosis is established against the background of a normal level of lymphocytes in the blood.

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

Pediatric agranulocytosis with cyclic neutropenia

Cyclic neutropenia is a disease that is hereditary. Its occurrence provokes an autosomal dominant type of inheritance of symptoms: the child's disease appears only in the case of at least one of the parents of a similar pathology.

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

Symptoms of cyclic agranulocytosis are recurrent fever, ulcerous lesions of the oral cavity and larynx, an increase in regional lymph nodes and obvious symptoms of intoxication. When a fever releases a child, his condition returns to normal. But some small patients receive instead of high temperature complications in the form of otitis, abscessed pneumonia, and so on.

The most important sign of cyclic neutropenia is the periodic decrease in the number of granulocytes in the blood plasma. In its most extreme stage appears agranulocytosis, which lasts for three to four days. After that, neutrophils begin to appear again in the blood, but in small amounts. In this case, an increase in the number of lymphocytes is possible. After this peak phase, in laboratory studies, transient monocytosis and eosinophilia can be identified. And then before the next cycle, all blood counts are within the normal range. In some patients with agranulocytosis, a decrease in the level of erythrocytes and platelets is observed.

Therapy of this disease in children is, first of all, in preventing complications from getting into the body of infections. In addition, the preparation of granulocyte colony-stimulating factor (G-CSF), which reduces the degree of granulocytopenia, has proven itself. Unfortunately, this medication can not prevent the appearance of cyclic changes in blood composition in the child.

Agranulocytosis in newborns with isoimmune conflict

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

The mechanism of this pathology consists in the formation in the mother's body of antibodies to children's granulocytes, which have the same antigenic composition as that of the baby's father. Antibodies are sent to the precursor cells of granulocytes, which produce red bone marrow.

Features of this type of agranulocytosis are manifested in the transit character of the disease. In this case, maternal antibodies are rapidly washed out by the fluids from the child's body at a high rate, which stimulates spontaneous normalization of the granulocyte count in the blood plasma of the patient. Therefore, the child recovers in ten or twelve days after the onset of the disease. At this time it is important to use antibiotics to prevent infectious complications.

[11], [12], [13], [14]