Aplastic anemia

  1. What kind of disease is aplastic anemia?  A: Aplastic anemia (AA), commonly known as “soft cancer”. This article mainly focuses on acquired reblastosis, which is a group of syndromes caused by multiple causes or unexplained bone marrow hematopoietic failure (bone marrow failure), and a lot of clinical and experimental data show that acquired reblastosis is an autoimmune disease. It is characterized by (1) a decrease in whole blood cells with corresponding clinical symptoms, such as leukopenia in which the patient is prone to infection and fever; erythrocyte reduction in which palpitations, shortness of breath, weakness and dizziness are seen; and platelet reduction in which bleeding is seen. (2) Low bone marrow proliferation, reduced hematopoietic tissue and increased non-hematopoietic tissue. (3) No enlargement of liver and spleen lymph nodes. (4) Commonly used anti-anemia drugs are ineffective.  2. What factors can cause aplastic anemia?  A: Most patients with aplastic anemia have an unknown cause, called primary or idiopathic aplastic anemia. Some patients are apparently caused by the toxic effects of chemical, physical or biological factors on bone marrow, which is called secondary reoccurrence. Secondary reoccurrence is mainly due to drugs, chemical toxicants benzene and its derivatives, ionizing radiation, and viral infections. Among the drug-induced reoccurrence, the most common one is chloramphenicol (co-trimoxazole), antipyretic and analgesic drugs such as anandamide and pautazone, antineoplastic drugs, sulfonamides (such as cotrimoxazole) and antibiotics such as vancomycin and voriconazole, etc. Drugs may also cause reoccurrence, and even penicillin can occasionally cause reoccurrence, although it is rare. Benzene contained in petroleum, gasoline, paints, plastics, and hair dyes can easily accumulate in the bone marrow and is clearly related to the occurrence of reoccurrence. Pesticides such as “666” and organophosphorus can also cause reoccurrence. Various kinds of ionizing radiation, such as X-rays, γ-rays and radionuclides, can cause reoccurrence when the dose reaches a certain level. Viral hepatitis can also occur in the later stages of the disease, and is often more serious. The primary reoccurrence reported in the literature, although the cause is unknown, there may actually be undiscovered causative factors.  3. What is the incidence of aplastic anemia?  A: In clinical practice, aplastic anaemia is divided into acute aplastic anaemia and chronic aplastic anaemia according to the urgency of its onset, severity of the disease and the degree of bone marrow damage. Depending on the cause, there are two types of reoccurrence: congenital and acquired. A congenital reoccurrence called Fanconi anemia accounts for 2 or 5% of reoccurrence, which usually develops within 10 years of age and most of them have family history. Acquired reoccurrence with unknown cause is called primary reoccurrence, accounting for about 70,3%, while those who can identify the cause are called secondary reoccurrence, accounting for 16,9%. In recent years, secondary reoccurrence has increased significantly. The incidence of reoccurrence can occur at all ages, but it is more common in young adults, with the peak age of incidence between 20 and 25 years old, more men than women in the Far East, and more in northern than southern China. The incidence rate in China is 7.4 per 100,000, of which 6.0 per 100,000 for chronic reoccurrence and 1.4 per 100,000 for acute reoccurrence. the incidence rate of reoccurrence in Asian residents (especially in the Far East), including China, is much higher than that of European and American residents (including Asians).  4. What are the clinical manifestations of aplastic anemia?  A: There are differences in the clinical manifestations of different types of reblastosis. Acute type of reblastosis, also known as heavy reblastosis type I. It starts rapidly, progresses rapidly, and often has bleeding and infectious fever as the first symptoms. Extensive and severe skin bleeding, gum bleeding, nasal bleeding, gastrointestinal bleeding, blood in the urine, fundus bleeding, and in severe cases, intracranial bleeding may occur. Most patients may see persistent high fever due to infection, with pharyngeal infection, pneumonia, perianal infection abscesses and resulting sepsis predominating. As the disease progresses, the anemia worsens progressively. This type of disease is serious and has a high mortality rate without treatment.  Most patients are seen for anemia, weakness, and frequent petechiae on the extremities. Most patients are seen for anemia, malaise, and frequent petechiae on the extremities. There is usually no infectious fever or only a mild irregular low-grade fever. The duration of the disease is more than 4 years, and can even be as long as 10 years. If treated properly and persistently, most of them can be cured, but there are patients who do not recover for many years. A small number of patients can have acute attacks, and the condition can take a sharp turn, which is called heavy remitting disease type II, often related to infection.  5.How to diagnose and differential diagnosis of aplastic anemia?  A: Aplastic anemia lacks specific clinical and laboratory manifestations. In a sense, the diagnostic criteria for aplastic anemia can in fact be understood as exclusion criteria for all other known bone marrow hematopoietic failures. The International Study Group on Granulocytopenia and Aplastic Anemia (1987) proposed that the diagnosis of aplastic anemia [1] must meet at least 2 of the following 3 points: (1) hemoglobin <100 g/L; (2) platelets <50 × 109/L; and (3) neutrophils <1,5 × 109/L. If a patient has a reduction in peripheral blood secondary or tertiary blood cells that does not meet these criteria, the patient should not be be diagnosed as repletion, but changes in blood cell counts need to be monitored closely. After the diagnosis of reocclusion, the clinical type should be further determined. At present, the international standard of Camitta (1976) is commonly used to classify reocclusion into heavy (SAA) and non-heavy (NSAA), and the diagnostic criteria of very heavy reocclusion (VSAA) were added in 1988 (Table 1).  In 1987, the Fourth National Conference on Recurrent Disorders formulated the diagnostic criteria for recurrent disorders in China, which have been used since then. Compared with the international diagnostic staging criteria for reoccurrence, our criteria, in addition to emphasizing blood and bone marrow examination, also incorporated clinical manifestations into the aplastic anemia staging criteria and staged them as acute aplastic anemia and chronic aplastic anemia. There is a high degree of agreement between domestic typing and Camitta typing, with the latter emphasizing the severity of hematopoietic failure and the former emphasizing the rapidity of the development of this failure in addition to the severity of hematopoietic failure. From the perspective of a comprehensive understanding of the disease, domestic staging has its unique advantages.  It is important to point out that the peripheral blood triad of peripheral blood cells shows a parallel decline in typical reblasts, but in some special cases, such as early reblasts, this feature may not be obvious and often manifests first as thrombocytopenia and neutropenia. Patients with anemia who still have normal platelet counts should be aware that they may have other disorders than reoccurrence; absolute peripheral reticulocyte counts in patients with reoccurrence should be emphasized. In patients with anemia, peripheral blood reticulocyte counts of at least 100×109/L should be considered as effective bone marrow compensation, while in patients with reoccurrence, bone marrow hematopoiesis is not compensated and reticulocyte counts are relatively or absolutely reduced; it is often unreliable to diagnose or exclude reoccurrence based on the results of a single bone marrow aspiration smear from a particular site, and the results of multiple bone marrow aspirations from different sites can more objectively reflect systemic bone marrow hematopoiesis; modern diagnosis of reoccurrence must The modern diagnosis of reoccurrence must include bone marrow biopsy to evaluate the area of bone marrow hematopoiesis and to make the necessary differential diagnosis; the evaluation of hypoplasia and steatosis of bone marrow must take into account the influence of age; the evaluation of hypoplasia of bone marrow in the elderly must be performed simultaneously with peripheral blood examination or multiple bone marrow aspirations at multiple sites; the typing diagnosis of reoccurrence by a single peripheral blood test is often unreliable and should be based on a dynamic, developmental perspective. A dynamic, developmental perspective should be used to predict disease progression and make a reasonable staging diagnosis early, especially in acute reoccurrence.  Other disorders causing allogeneic cytopenia, such as (1) acute hematopoietic arrest (AHA), (2) myelodysplastic syndrome (MDS), (3) Fanconi anemia (FA), (4) paroxysmal sleep hemoglobinuria (PNH), (5) Evans syndrome, (6) immune-related allogeneic cytopenia (IRP), (7) myelofibrosis (MF), (8) hairy cell leukemia (HCL), (9) hypoproliferative leukemia, (10) mesenchymal T-cell lymphoma, etc.  6. How is aplastic anemia treated?  A: Western medical treatment for aplastic anemia mainly includes the following: (1) androgens. ② Immunosuppressants, such as cyclosporine A, ATG (horse ATG is better than rabbit ATG), high-dose methylprednisolone, high-dose propecia, etc. ③Improve microcirculation such as 654-2, etc. ④Chinese medicine believes that the pathogenesis of reoccurrence is related to heart, liver, spleen and kidney, especially the relationship between kidney and hematopoiesis is the closest. Chinese medicine has been proven to have the ability to promote bone marrow stem cell differentiation and improve the immunity of the body, therefore, it is now recognized that the application of combined Chinese and Western medicine treatment has significantly improved the efficacy, and the effective rate of acute and chronic reoccurrence is over 90%, and the recovery rate is 60-70%. Allogeneic hematopoietic stem cell transplantation: After AA treatment, platelet recovery is usually slower than that of leukocytes.  7.What is the prognosis of aplastic anemia?  A: Before the 1990s, the mortality rate within one year was almost 100% for acute reblastosis. With the progress of medical science, most of the patients can be reborn, and most of them can be cured or basically cured as long as they adhere to the systematic treatment.  Of course, we should also be aware that the treatment process of reoccurrence is relatively long, and there are many complications during the process, and heavy reoccurrence will only be effective after at least 3 months of treatment. As medical personnel, although we are full of confidence, we must have the close cooperation of patients' families. Only when both doctors and patients cooperate well, communicate with each other, understand each other and work together for a common goal, we can finally reach the other side of victory and return you a healthy relative and a beautiful family.