lazy leukocyte syndrome
Introduction
Introduction to lazy white blood cell syndrome Lazy-leukocytesyndrome, also known as neutrophilparalysis, is caused by neutrophil chemotaxis and insufficiency of granulocyte function, resulting in repeated infections. More common in children. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: sinusitis otitis media pneumonia
Cause
Lazy white blood cell syndrome
(1) Causes of the disease
It is currently believed that its cause is a defect in the gene. Secondary neutrophil paralysis is found in rheumatoid arthritis, systemic lupus erythematosus, uremia, severe infection, myeloma, cirrhosis, graft-versus-host response, immune complexes, Severe eczema and other diseases, granulocyte palsy may be related to blood immunoglobulins, immune complexes, bacterial endotoxin adsorption on granulocyte membranes, diabetic ketoacidosis or high blood sugar may be due to excessive blood osmotic pressure.
(two) pathogenesis
Neutrophil chemotaxis dysfunction affects its migration from the bone marrow to the peripheral blood, granulocytes are reduced, and actin monolayers in neutrophils cannot aggregate to form fibrils, resulting in two defects: 1 can not form pseudopodia; 2 can not regulate the fusion of particles and membrane, the patient's neutrophils are normal in morphology and oxygen metabolism, but will not move to the inflamed site, phagocytic function is also poor, but in the case of engulfing It can release too much enzyme from the particles to the extracellular and phagosome, and the patient's monocyte function is normal.
In addition, some people think that the granulocyte migration function of patients with this disease is abnormal, may be due to the structural or functional abnormality of the microfilamentous protein of the granulocyte membrane, resulting in cell membrane stiffness, not easily released from the bone marrow into the blood circulation, or recycled to the tissue. .
Prevention
Lazy white blood cell syndrome prevention
The disease is a defective disease of the gene, and the cause is not clear. It may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy. Therefore, the disease cannot be directly prevented. Early detection, early diagnosis, and early treatment are important for preventing this disease. Regular examination should be carried out during pregnancy. If the child has a tendency to develop abnormalities, abortion should be carried out in time to avoid the birth of the child.
Complication
Lazy white blood cell syndrome complications Complications sinusitis otitis media pneumonia
The main complications are: refractory infections, such as stomatitis, sinusitis, otitis media, pneumonia, skin infections, etc., can be repeated; peripheral blood leukocyte count can be slightly decreased, neutrophils are significantly reduced, lymphocytes are relatively increased Primary neutrophil paralysis is found in rheumatoid arthritis, systemic lupus erythematosus, uremia, severe infections, myeloma, cirrhosis, graft versus host response, immune complexes, severe eczema and other diseases.
Symptom
Lazy white blood cell syndrome symptoms common symptoms gingivitis
Gram-positive or negative bacterial infections, such as otitis media, stomatitis, gingivitis and hypothermia, occur after birth, and the infection affects the skin and gastrointestinal tract, but does not purulent.
Examine
Lazy white blood cell syndrome check
Peripheral blood
The total number of white blood cells and neutrophils decreased, and lymphocytes increased relatively.
2. The bone marrow is normal.
3. The skin window experiment showed no neutrophil aggregation, adhesion and phagocytosis, and cortisol was applied.
4. Adrenaline, injection of bacterial heat source can not promote the increase of granulocytes, indicating the storage of granulocyte release disorders, abnormal granulocyte migration.
5. Immunoglobulins are normal.
According to the condition, clinical manifestations, symptoms, signs, X-ray, B-ultrasound, biochemical examination.
Diagnosis
Diagnosis and identification of lazy white blood cell syndrome
1 refractory infections: such as stomatitis, sinusitis, otitis media, pneumonia, skin infections, etc., can be repeated;
2 peripheral blood leukocyte counts can be slightly decreased, neutrophils are significantly reduced, and lymphocytes are relatively increased;
3 Adrenalin test can not increase the neutrophils of peripheral blood;
4 neutrophil chemotactic movement and deformation movement weakened, phagocytic function and intracellular bactericidal function were normal;
5 bone marrow is normal;
6 Body fluid and cellular immune function tests were normal.
Identify through the above aspects.
