neutropenia
Introduction
Introduction to neutropenia Neutrophilic granuloytopenia is the absolute count of peripheral blood neutrophils (total number of white blood cells × percentage of neutrophils), less than 1.5 × 109 / L for children <10 years old, lower than children aged 10 to 14 years old 1.8 × 109 / L, less than 2.0 × 109 / L in adults, when granulocytes are severely reduced, below 0.5 × 109 / L, called agranulocytosis. basic knowledge The proportion of sickness: 0.01% Susceptible people: no specific population Mode of infection: non-infectious Complications: agranulocytosis, sepsis
Cause
Cause of neutropenia
Genetic factors (25%):
Certain congenital and hereditary neutropenia: such as Kostmann syndrome, reticular hypoplasia with congenital leukopenia, neutropenia with abnormal granulocyte production, etc. 5 immune disorders: systemic Lupus erythematosus, rheumatoid arthritis, etc.; 6 infection: bacterial infections such as typhoid fever, paratyphoid fever, brucellosis, miliary tuberculosis; viral infections such as hepatitis, AIDS, etc.;
Other diseases (15%):
Blood diseases: such as bone marrow metastasis, myelofibrosis, lymphoma, leukopenia, aplastic anemia, multiple myeloma, malignant histiocytosis.
Acquired factors (10%):
Maturity disorders: 1 acquired: such as folic acid deficiency, vitamin B12 deficiency, pernicious anemia, severe iron deficiency anemia, etc.; 2 malignant and other clonal diseases: such as myelodysplastic syndrome, paroxysmal nocturnal hemoglobinuria, etc. .
Drug factors (10%):
Including cytotoxic and non-cytotoxic drugs;
Physical and chemical factors (5%):
Radiation, chemical substances: such as benzene, DDT, dinitrophenol, arsenic acid, cesium, nitric oxide, etc.;
Pathogenesis
The pathogenesis of neutropenia is nothing more than:
1 granulocyte production is reduced or ineffective;
2 granulocytic destruction is excessively lost, and granulocyte lifespan is shortened;
Abnormal distribution of 3 granulocytes;
4 Integrate the first three mechanisms.
Prevention
Neutropenia prevention
Strict protection system should be established for close contact with radiation, benzene or other harmful substances, and regular blood tests should be carried out for timely diagnosis and treatment. For patients using cytotoxic drugs, the number of granulocytes should be measured every 1-2 days. Timely reduction of drug dose or withdrawal, as far as possible to avoid or limit the use of drugs that may cause neutropenia, when taking drugs that may cause neutropenia (non-cytotoxic drugs), you should pay attention to weekly blood tests, allergic to drugs Those with history or drug-induced neutropenia or lack of medical history should avoid taking the same and similar drugs.
Complication
Neutropenia complications Complications, agranulocytosis, sepsis
The main complication of neutropenia is infection, including lung infection, oral infection, skin infection, etc., such as agranulocytosis often sudden onset, patients have chills, high fever, the most prone to serious infections in the mouth, showing necrosis Ulcers, often covered with gray or black pseudomembrane, soft palate or pharyngeal arch can be perforated due to necrosis, the so-called walking horse, perineal infection is second only to the oral cavity, rectal, perianal and vaginal can occur Necrotic ulcers, if the infection of the above-mentioned parts is not treated in time, the lack of granulocytes often causes the infection to spread rapidly, progressing to sepsis, and the mortality rate is high.
Symptom
Symptoms of neutropenia Common symptoms Weak leukopenia Reduce fungal infections Granulocyte reduction
Granulocyte dysfunction is a hematological abnormality. If there is no infection, there is often no clinical manifestation, such as long-term neutropenia. Some patients may complain of fatigue and drowsiness. Once infected, the corresponding symptoms may occur depending on the infected site. And signs.
1. The number of leukopenia in peripheral blood leukocytes is <4×109/L, and children are determined by reference to normal values of different ages.
2. Apoptotic peripheral blood ANC < 1.5 × 109 / L.
3. Agranulocytosis peripheral blood ANC < 0.5 × 109 / L.
In short, after the above diagnosis is determined, the primary disease should be detected according to the diagnostic criteria of various primary diseases, and the infected person should be clearly defined according to the diagnostic criteria of various infections.
Examine
Examination of neutropenia
1. Blood: The blood cell count usually shows a decrease in the number of white blood cells. The granulocytes in the classification are significantly reduced, while the lymphocytes are relatively increased. If the microscope can be directly observed, it is more reliable and correct. A few patients may be accompanied by anemia and/or thrombocytopenia.
2. Bone marrow: Most of the bone marrow hyperplasia is in the normal range, but the granulocyte hyperplasia is often reduced, accompanied by maturity disorders, ie, the neutrophils below the late granules, the erythroid and megakaryotypes are basically normal. For various infections that cause neutropenia, the necessary laboratory tests should be selected to confirm the diagnosis.
3. Antigen and antibody detection : The most common viral infections should be tested for antigens and/or antibodies related to the virus. Bacterial and fungal infections should be taken from the secretion secretion culture and blood culture. It also has important value.
4. Imaging examination of infected lesions : It can locate the diagnosis and help to determine the qualitative. Patients with malignant tumors can be identified by various related imaging examinations, and pathological or cytological examination can confirm the diagnosis.
5. Autoantibodies: The detection of autoantibodies in patients with autoimmune diseases is the key to diagnosis.
Diagnosis
Diagnosis and diagnosis of neutropenia
diagnosis
According to the results of the examination can be diagnosed.
Diagnostic evaluation: The diagnosis of neutropenia is usually without difficulty. It can be determined according to the blood test. Because it is only a hematological abnormality in most cases, it is not an independent disease. Therefore, the difficulty in diagnosis is to find the original. disease.
After neutropenia is often associated with infection, it is still necessary to identify whether the infection is a primary disease, or a complication, sometimes it is difficult. Clinically secondary neutropenia is most common in viral infection, because most patients do not have neutropenia. It usually does not contain serious bacterial or fungal infections, so its clinical significance is limited, and often does not require special treatment. What is really clinically significant is neutropenia, almost all of which are infected, requiring immediate emergency treatment, in which the drug passes the immune mechanism. The inducer is the first, most of which is determined by the individual's specific constitution, so it can't be prevented. It can be expected to take control dosage, medication time and G-CSF prevention, which is easier to solve. In addition, some occur in infants. Congenital agranulocytosis in young children and neonatal period is fierce and needs to be judged and dealt with in time.
Differential diagnosis
1. The causes of secondary neutropenia identify the above eight categories of causes, according to the history and clinical manifestations are generally not difficult to identify, but drug-induced, sometimes determined to be caused by a certain drug, there will be some difficulty, because patients may At the same time or in succession of multiple drugs, the significance of identifying which drugs should be banned in the future.
2. Identification of various congenital neutropenia according to the degree of neutropenia, can be identified as benign or severe neutropenia; periodic regular author, the most easy to confirm the diagnosis; granulocytes associated with various syndromes If it is reduced, it can be distinguished according to the clinical characteristics and laboratory results. In addition, the age of onset has certain reference significance for diagnosis.
3. Identification of granulocytopenic infection and infection-induced neutropenia Non-infectious causes of neutropenia, neutropenia occurs before infection, and infection is complicated; infection-induced neutropenia, vice versa The neutropenia caused by infection, such as concurrent infection, mostly has a clear primary disease.
4. Recovery period of agranulocytosis and acute leukemia identify patients with recovery of granulocytosis, mainly bone marrow (sometimes involving blood) can appear more early granulocyte, including primitive and promyelocytes, sometimes more than 30 %, resembles acute myeloid leukemia, identification point:
1 The former has a history of agranulocytosis, and a history of primary disease or medication,
2 no signs of lymph node, liver, spleen and other leukemia infiltration,
3 usually without anemia and/or thrombocytopenia,
4 Short-term dynamic observation, early granulocyte in bone marrow and peripheral blood gradually decreased and disappeared.
