Alloimmune, or isoimmune, neutropenia of newborns
Last reviewed: 23.04.2024
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The incidence of alloimmune or isoimmune neutropenia in newborns is 2 cases per 1000 live births.
Alloimmune, or isoimmune, neutropenia of newborns occurs in the fetus due to the antigenic incompatibility of the neutrophils of the fetus and the mother. The isoantibodies of the mother belong to the IgG class, they penetrate the placental barrier and destroy the child's neutrophils. Isoantibodies are usually leukoagglutinins, they react with the cells of the patient and his father, do not react with the cells of the mother.
Alloimmune, or isoimmune, neutropenia of newborns is diagnosed during the period of the newborn and the first 3 months of life. The diagnosis is confirmed by the presence of isoantibodies in the serum of the patient.
Criteria for diagnosis of autoimmune neutropenia:
- autoantigranulocyte antibodies in the patient's blood serum;
- communication of neutropenia with transferred infectious (often viral) diseases and / or taking medications (sulfonamide, NSAIDs, etc.);
- an increase in the content of plasma cells in peripheral blood and an inverse correlation of their number with the number of neutrophils;
- spreading of the immune conflict to other blood cells.
The main criterion is autoantibody to neutrophils.
In young children, acute moderate forms of autoimmune neutropenia predominate. With this option, the number of leukocytes, platelets, red blood cells correspond to the norm, absolute neutropenia is 0.5-1.0x10 9 / l. Iron deficiency anemia can be observed. In the myelogram the neutrophilic germ corresponds to the norm or increases, the number of stab and segmented neutrophils decreases, and the lymphocyte content may increase. The remaining parameters of the bone marrow correspond to the norm.
Treatment of infection with alloimmune, or isoimmune, neutropenia of newborns is carried out by conventional means. In moderate forms, the use of immunoglobulins (IgG) for intravenous administration (IVIG) is indicated, with rare severe - granulocyte colony-stimulating factor in combination with IVIG and broad-spectrum antibiotics. In the absence of infections, treatment is not carried out. The disease is self-inflicted to 3-4 months of age. Preventive vaccinations are performed one year after the remission is achieved in full.
Treatment of autoimmune neutropenia is determined by the severity of the condition and the variant of the course. In mild forms, special treatment is not carried out. For moderate to severe and severe autoimmune neutropenia, glucocorticosteroids are used at a dose of 2-5 mg / kg per day, normal human immunoglobulin (IVIG - octagam, sandoglobulin, biaven) at a course dose of 1.5-2 g / kg. The drugs of choice are the growth factors of the granulocyte colony-stimulating factor at a dose of 8-10 μg / kg per day in combination with basic therapy (antibacterial, antifungal and / or antiviral). In chronic autoimmune neutropenia, granulocyte colony-stimulating factor and IVIG are effective. In children with autoimmune neutropenia, the question of carrying out preventive vaccinations is decided depending on its cause. After acute primary autoimmune neutropenia, an easy form of vaccination should be postponed for up to 1 year. In severe forms and chronic course, the tactics are individual.
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