Introduction to neutropenia

Introduction Neutrophilic granuloaytopenia is the absolute count of peripheral blood neutrophils (total number of white blood cells × percentage of neutrophils), children <10 years old are less than 1.5 × 109 / L, children aged 10 to 14 years old Below 1.8 x 109 / L, adults below 2.0 × 109 / L. When granulocytes are severely reduced, below 0.5 × 109 / L, called agranulocytosis. Causes (1) Causes of neutropenia caused by many causes, according to various reasons, can be summarized as follows: 1. Acting on bone marrow (1) Bone marrow damage: 1 drug: including cytotoxicity And non-cytotoxic drugs; 2 radiation; 3 chemicals: such as benzene, DDT, dinitrophenol, arsenic acid, strontium, nitric oxide, etc.; 4 certain congenital and hereditary neutropenia: such as Kostmann synthesis Signs, reticular hypoplasia with congenital leukopenia, neutropenia with abnormal granulocyte production, etc.; 5 immune disorders: such as systemic lupus erythematosus, rheumatoid arthritis, etc.; 6 infection: bacterial infection Such as typhoid fever, paratyphoid fever, brucellosis, miliary tuberculosis; viral infections such as hepatitis, AIDS, etc.; 7 blood diseases: such as bone marrow metastasis, myelofibrosis, lymphoma, leukopenia, aplastic anemia , multiple myeloma, malignant histiocytosis, etc. (2) 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 hemoglobin Urine and so on. 2. Acting on peripheral blood (1) neutrophil circulatory pool switch to marginal pool (ie pseudoneutropenia): 1 hereditary benign pseudoneutropenia; 2 acquired: as serious Bacterial infections, malignant malnutrition, malaria, etc. (2) Intravascular detainment: such as detaining in the lung caused by complement-mediated leukocyte lectin, detaining in the spleen caused by hypersplenism. 3. Acting on the extravascular (1) Increased use: such as serious bacteria, fungi, viruses or rickettsial infections, allergic diseases, etc. (2) Increased damage: such as hypersplenism. (B) the pathogenesis of neutropenia pathogenesis is nothing more than: 1 granulocyte production decreased or ineffective generation; 2 granulocytic destruction lost too much, granulocyte lifespan shortened; 3 granulocyte distribution abnormal; 4 integrated before 3 Kind of mechanism. Symptoms of neutropenia are a hematological abnormality, and if there is no infection, there is often no clinical manifestation. Such as long-term neutropenia, some patients can complain of fatigue, sleepiness. Once the infection is combined, the corresponding symptoms and signs appear depending on the infection site. 1. Leukopenia peripheral white blood cell count <4 × 109 / L. 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 determining the above diagnosis, it is necessary to detect the primary disease according to the diagnostic criteria of various primary diseases. Patients with concurrent infections are clearly defined according to the diagnostic criteria for various infections. Diagnostic evaluation: The diagnosis of neutropenia is usually without difficulty and can be determined according to the blood test. Because it is only a hematological abnormality in most cases, it is not an independent disease, so the difficulty in diagnosis is to find the primary disease. After neutropenia is often combined with infection, it is still necessary to identify whether the infection is a primary disease, or a complication, sometimes difficult. Clinically secondary neutropenia is most common in viral infections. Because most patients do not have granulocytopenia, they usually do not have serious bacterial or fungal infections, so their clinical significance is limited and often requires no special treatment. What is really clinically significant is neutropenia, almost all of which are infected, requiring immediate emergency treatment. Among them, drugs are the first to be induced by immune mechanism, and most of them are determined by individual physique, so they cannot be prevented. Caused by cytotoxic drugs, it can be expected to take control dosage, medication time and prevention of G-CSF, which is easier to solve. In addition, some congenital agranulocytosis in infants and neonates is fierce and needs to be judged and dealt with in a timely manner. Diagnosis of neutropenia needs to be identified from the following aspects. 1. The etiology of secondary agranulocytosis identifies the above eight categories of causes, which are generally not difficult to identify based on medical history and clinical manifestations. However, drug-induced, sometimes determined to be caused by a certain drug, will be difficult because the patient may use multiple drugs at the same time or in succession. The significance of identification is which drugs should be banned for life 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 Reduced, can be distinguished according to their clinical characteristics and laboratory results. In addition, the age of onset has a 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 concurrent; infection-induced neutropenia, vice versa. Non-infectious causes of neutropenia, such as concurrent infections, mostly have 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 points: 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. The main complication of complication of neutropenia is infection, including lung infections, oral infections, skin infections, and the like. Such as agranulocytosis often sudden onset, patients have chills, high fever, the most prone to serious infections in the oral cavity, manifested as necrotizing ulcers, often covered by gray or black pseudomembrane, soft palate or pharyngeal arch can be perforated due to necrosis, ie The so-called walking. Infection in the perineum area is second only to the prone area of the oral cavity. Necrotic ulcers can occur in the rectum, perianal and vagina. 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. Treatment (1) Treatment of neutropenia is mainly caused by etiological treatment, such as discontinuation of suspicious drugs, cessation of contact with suspected toxicants, and treatment of various primary diseases that cause neutropenia. The main manifestation of neutropenia is infection, but the risk of infection is negatively correlated with the degree of neutropenia: patients with neutrophils (1.0 to 1.8) × 109 / L are less likely to develop infection. , (0.5 ~ 1.0) × 109 / L is centered, <0.5 × 109 / L patients are the most likely. In addition, the frequency and severity of infections are related to the cause and duration of neutropenia. In general, neutrophil production reduces disease, such as secondary to cytotoxic drugs, bone marrow radiotherapy, or early hematopoietic progenitor cells. Patients with neutropenia caused by defects, etc. have a greater chance of infection than patients with other causes; neutropenia is accompanied by mononuclear cell reduction, lymphopenia, hypogammaglobulinemia Patients with symptoms are more severe than those with only simple neutropenia. Therefore, the treatment of neutropenia should vary from person to person and from disease to disease. 1. Treatment of neutrophil counts (1) Leukocyte-producing drugs: many clinical applications, such as vitamin B6, vitamin B4, blood, inosine, deoxynucleotides, androgens, lithium carbonate Etc., but lack of affirmative and long-lasting efficacy, therefore, the initial treatment of patients can choose 1 to 2, every 4 to 6 weeks to replace a group, until effective, if it does not work for several months, do not have to continue to use. Read more...