Pediatric pseudoneutropenia
Introduction
Introduction to pediatric pseudoneutropenia In patients with pseudoneutropenia (Pseudoneutropenia), the granulocyte fluctuations in the surrounding blood range are large, and the changes are rapid, and many serious infections are not combined. This is caused by abnormal granulocyte distribution. When the blood flow velocity is slow, granulocytes tend to adhere to the small blood vessel wall, resulting in increased granulocytes in the marginal pool. After injection of adrenaline, granulocytes can be raised to normal or rise to One to two times before the test can be used as a basis for diagnosing the disease. Patients with only neutropenia, no primary disease and no recurrent infection can be collectively referred to as benign neutropenia, including familial, congenital and pseudoneutropenia, intermittent, granulocytes The degree is reduced and the process is good. basic knowledge The proportion of illness: 0.012% Susceptible people: children Mode of infection: non-infectious Complications: sepsis
Cause
Causes of pediatric pseudogranulocytopenia
(1) Causes of the disease
Some families have found autosomal dominant genetic predispositions, such as amino-based drugs or radiation-induced damage; mood changes, exercise, and changes in body temperature can lead to abnormal granulocyte distribution.
(two) pathogenesis
The distribution of circulating granulocytes is abnormal. Only 1/2 of the neutrophils entering the blood vessels are in the circulation pool, that is, they circulate with the blood flow, and another 1/2 of the neutrophils are in close contact with the capillaries and the capillaries. Endothelial cells (edge cells) do not circulate with the blood flow, so they cannot be detected at the time of white blood cell count. The granulocytes between the circulation pool and the edge pool can be converted to each other. Under the condition of injection of adrenaline or stress, granulocytes can be rapidly formed by the edge pool. Transfer to the circulation pool, the granulocyte count is significantly increased, such as the relative increase of granulocyte volume in the marginal pool can cause pseudo-granulocyte reduction, granulocyte production and utilization are normal, systemic infection and allergic reaction can cause reaction Sexual acquired pseudogranulocyte deficiency, white blood cells in the infection, whether it is chemotaxis or phagocytosis is relatively slow, so it is difficult to play a protective role for severe suppurative infection.
Prevention
Pediatric pseudoneutropenia prevention
Often family history, should be consulted with genetic diseases, but also to prevent predisposing factors, such as granulocyte reduction caused by drugs, should stop immediately, stop exposure to radiation or other chemical poisons; prevent secondary infections, take preventive measures, If you do not measure the body temperature of the anus. Patients who do not have fever should not abuse antibiotics. Pay attention to the oral cleansing of children and prevent intestinal infections. All foods and utensils should be disinfected and vaccinated.
Complication
Pediatric pseudoneutropenia complications Complications sepsis
Often complicated by various infections, the infection is easy to spread quickly, progressing to sepsis, serious infection is an important cause of death in children.
Symptom
Symptoms of Pseudogranulocyte Deprivation in Children Common Symptoms Granulocyte Depletion Lack of Leukopenia Depression, Dizziness, Discomfort, Recurrent Sudden Infection, Throatitis, Hypothermia
The neutropenia caused by various reasons has similar clinical symptoms, which is related to the degree of neutropenia. The onset can be acute and slow. Most patients with leukopenia are often short-lived and have no clinical manifestations. Symptoms or dizziness, fatigue, hypothermia, pharyngitis and other non-specific manifestations, neutrophils are the first line of defense against infection, so the clinical symptoms of neutropenia are mainly susceptible to repeated infections, infections in patients The risk is directly related to the neutrophil count, the time of reduction and the rate of decrease. The neutrophils are significantly reduced, down to 1.0×109/L, and even completely absent, which is completely different from the general leukopenia. Sudden onset, patients can suddenly chills, high fever, sweating, general discomfort, almost all within 2 to 3 days of serious infections, more complicated lungs, urinary tract, skin, oral mucosa and skin and other serious infections or sepsis Staphylococcus aureus, Escherichia coli and Pseudomonas aeruginosa are the most common, and the mucosa may have necrotizing ulcers.
Due to the lack of granulocytes that mediate the inflammatory response, the signs and symptoms at the time of infection are usually not obvious; such as severe pneumonia, only mild infiltration and no purulent sputum on the chest radiograph; severe skin infection does not cause bloating; pyelonephritis does not see pyuria Etc., the infection is easy to spread quickly, progress to sepsis, the mortality rate is very high, acute cases can often find the cause, such as amino-based drugs or radiation damage, stop the drug after rescue treatment, body temperature returns to normal, Peripheral blood leukocytes rise, indicating that the condition is getting better. If the infection is not controlled in time, it can cause death. The onset of the disease is often chronic, with intermittent episodes. The moderate reduction of granulocytes is accompanied by low fever and fatigue, due to the compensatory function of the body. Some patients have no obvious symptoms or infection, and their blood features are more than 1.0×10 9 /L, and there is a compensatory increase in monocytes, but mononuclear cells are in the process of infection. Chemotherapy or phagocytosis is slower than granulocytes, so it is difficult to protect the severe suppurative infection. The general process is good, with age, can be OK eased.
Examine
Examination of pediatric pseudogranulocytopenia
1. Blood: The total number of white blood cells and the percentage of neutrophils decreased (the total number of white blood cells in the decimal type is normal), ANC (the total number of white blood cells × the percentage of neutrophils) is lower than the normal level, red blood cells, hemoglobin, platelets are generally normal, white blood cell classification may have Monocytes, eosinophils, and lymphocytes are slightly increased. Before the ANC returns to normal, immature granulocytes may appear first, and even leukemia-like reactions may occur. Once neutropenia is found, it should be monitored 2 to 3 times a week. If necessary, it should be continuously monitored for 6-8 weeks to determine whether it is periodic or persistent. The peripheral blood white blood cell count is lower than 4.0×109/L (4000/mm3), called leukopenia, and leukopenia is the most common. It is caused by neutropenia, and the absolute count of neutrophils is lower than (1.8~2.0)×109/L (18002000/mm3), called neutropenia, lower than (0.51.0). ) × 10 9 /L (500 ~ 1000 / mm3) is called agranulocytosis, often accompanied by severe uncontrollable infections.
2. Bone marrow: Selective analysis of bone marrow, except for other diseases involving bone marrow hematopoiesis, and help to analyze the mechanism of neutropenia. In general, myeloid proliferation and cell formation are basically normal, more common in pseudo-granules. Cell reduction, hyperplasia is more common in immune granulocyte destruction or VB12, folate deficiency granulocyte ineffective production, granulocyte hyperplasia or original, promyelocytic cells decreased or cytoplasmic toxic particles found in drugs, chemistry Inhibition of granulocyte proliferation caused by poisons, etc., promyelocytic cells are normal and middle, and granulocytes are reduced in the late stage of late granulocyte, which is found in mature obstacles. Generally, erythroid cells and megakaryocytes are not significantly changed. Bone colonies are found in vitro culture of bone marrow stem cells. The number decreased, and the colony/plex ratio <1 indicated that the granulocyte hyperplasia was poor. In the culture system, the patient serum was added and the patient serum was not added. The number of colonies and the degree of differentiation were observed in the two groups to help to know whether there was inhibition of granules in the serum of the patients. Factors of proliferation, differentiation and maturation, when combined with infection, X-ray examination, B-ultrasound, etc. were selected according to clinical manifestations.
Diagnosis
Diagnosis and diagnosis of pediatric pseudogranulocytopenia
diagnosis
When the absolute value of granulocytes in childhood is less than 1.5×10 9 /L, the diagnosis can be diagnosed when the infant is less than 1.0×10 9 /L. However, the blood and bone marrow should be checked regularly. The total number of white blood cells can be checked and classified twice a week. 6 to 8 weeks to determine the degree of leukopenia, periodic or persistent reduction, to find the cause and judge the effect of treatment, and should ask in detail about the history and family history, especially the infection, clearly or not familial or Congenital factors, if necessary, do the following tests according to the condition to further find the cause.
1. Exercise test: If the granulocytes are reduced before exercise and increase to normal after exercise, the neutrophils attached to the blood vessel wall increase, which is pseudoneutrophil reduction.
2. Adrenaline test: subcutaneous injection of 0.1 adrenaline 0.1 ml, white blood cell count and classification before injection and 5, 10, 15 and 30 min after injection, if the granulocyte rises to normal after injection, indicating granulocyte reduction due to adhesion Increased granulocytes on the vessel wall; or due to enlarged spleen, the spleen contracts after administration and granulocytes rise.
3. Check the factors that destroy granulocytes: If it is suspected to be connective tissue disease or due to immunity, anti-nuclear antibodies should be tested, lupus cells should be searched, leukocyte agglutination test, leukocyte anti-human globulin consumption test, etc.
Differential diagnosis
When necessary, analyze the identification of bone marrow and leukemia and other myeloblastic granulocytopenia.
1. Bone marrow granulocyte production and maturation function: see bone marrow analysis and stem cell culture.
2. Bone marrow granulocyte reserve function or release function test: hydrocortisone or 5-androsterone (etiocholanolone) test, -androsterone can stimulate the release of mature granulocytes in the bone marrow storage pool, intramuscular injection 0.1mg/kg 14 ~18h, ANC rises up to 2.6 × 109 / L ~ 3.8 × 109 / kg or more.
3. Measurement of peripheral blood granulocyte distribution: Adrenalin can accelerate blood flow, and the number of granulocytes in the marginal pool increases into the circulating pool. The patient is injected subcutaneously with 1:1000 adrenal gland 0.1ml and then measured ANC at 5, 10, 15 and 30 min. , increased to normal or increased by 1 times than the original level, it is shown as pseudogranulocyte reduction.
4. Detection of factors that increase granulocyte destruction.
(1) It is suspected that the disease caused by immune destruction can selectively detect the presence of relevant anti-granulocyte-specific antibodies in the serum. Since the detection technology needs to be improved, anti-granulocyte antibodies are often not detected in many cases, so the negative ones The diagnosis cannot be denied.
(2) In some cases of leukocyte agglutination test, leukocyte lectin can be detected in the serum, but a person with multiple blood transfusion history may also be positive.
(3) Determination of serum lysozyme levels Neutrophils contain lysozyme, and serum lysozyme levels are elevated, suggesting that there may be an increase in peripheral blood granulocyte destruction.
5. Other suspects secondary to other primary diseases, may choose to do related tests, such as suspected and caused by antibodies caused by lupus erythematosus, the above 2, 3 tests have been used to assess bone marrow reserve function and peripheral blood edge pool Standard test for granulocyte response, but in recent years some people believe that the results are unstable, clinical application value is not large, has been used infrequently.
