1. Prognostic factors before treatment The prognosis of childhood acute lymphoblastic leukemia is better than that of adult acute lymphoblastic leukemia. There are many factors affecting the prognosis of childhood acute lymphoblastic leukemia. Therefore, it is advocated that ALL in children should be divided into standard risk group and high risk group according to different prognostic factors (Table 9). Some factors that have great influence on the prognosis of ALL in children mainly include: age, total white blood cell count, immunological and morphological classification of chromosome changes, and whether CNSL is combined.
(1) Age: Almost all the literatures confirm that age is an important independent factor. The prognosis of children aged 2- 10 months is good, the CR rate is 95%, EFS is over 70%, and the prognosis of infants younger than 12 months is very poor, which may be related to the chromosome T (4; 1 1) translocation is associated with acute heterozygous leukemia.
(2) Total number of white blood cells in peripheral blood: A large number of studies show that the total number of white blood cells has a very important influence on CR rate and EFS. The total number of white blood cells is the most critical factor affecting the prognosis of ALL in children. Generally speaking, white blood cells ≥50× 109/L are considered as high risk factors. No matter for ALL or AML with white blood cells higher than 100× 109/L, the CR rate is low, the survival time is short and the prognosis is poor in clinical treatment.
(3) Cytogenetic abnormality: With the development of chromosome banding technology, almost ALL studies have affirmed that chromosome abnormality is of great significance to the CR survival period of ALL and is a key index to judge high-risk factors. Children account for 5% of all chromosomal abnormalities in pH 1 (+). Their survival time is short and their prognosis is poor. The BCR-ABL fusion gene of patients is often higher than that of T (9; 22) Chromosome translocation is easier to find. t(9； 22) and t (4; 1 1) has a poor prognosis. Most t (4; 1 1)ALL has the immunophenotype of pre-B-ALL and can express myeloid antigen at the same time, suggesting that this kind of ALL may be an earlier source of stem cells.
(4) Immunotyping: Due to the changes of subtypes and prognosis caused by strong chemotherapy and the in-depth study of cytochrome technology, there are different reports on the relationship between immunophenotyping and prognosis. In the past, CD 10+ALL had a good prognosis. New research shows that the prognosis of CD 10+ mainly depends on whether it is accompanied by Ph 1(+) or BCR-ABL fusion gene. It is generally believed that early pre-B-ALL, C-ALL and pre-B-ALL subtypes have a better prognosis in children with ALL. Only 1% ~ 2% B-ALL is rare. Most of them have the morphological characteristics of L3 and the cytogenetic characteristics of Burkitt lymphoma. In the past, it was thought that the prognosis was poor and the long-term survival rate of children's B-ALL could reach more than 50% because of the great changes in the application of strong chemotherapy. T-ALL accounts for about 10% ~ 15% of ALL, which mostly occurs in older children over 10 years old, with high white blood cells and poor prognosis of mediastinal tumors. There are different opinions on the prognostic significance of ALL with myeloid antigen expression (My+). It is generALLy believed that the prognosis of all with myeloid antigen expression (My+) in children is not significant, but it is considered that the prognosis of all with CD34 expression is poor and needs further study and confirmation.
(5) Others: Lymph nodes in the liver and spleen are considered as the infiltrating sites of leukemia, and the prognosis is poor, but these factors are often influenced by factors such as abnormal age of cytogenetics and immune classification and white blood cells. It is generally believed that the combination of central nervous system or testicular involvement and mediastinal tumor at the time of initial diagnosis is an indicator of poor prognosis
According to the above scoring criteria, those with more than 3 points are high-risk ALL, and those with less than 3 points are standard risk ALL.
Among the above factors, the onset age and the total number of peripheral white blood cells are the most important prognostic factors. Studies have pointed out that these two factors account for 60% of the prognostic factors. If the white blood cell count is greater than 100× 109/L, it is often complicated with central nervous system leukemia; If the white blood cell count is greater than 50× 109/L and the age is less than 1 or greater than 10, the 4-year disease-free survival rate is only 64%.
2. Prognostic factors related to treatment In recent years, by summarizing all the curative effects of several centers, it was found that the therapeutic response was of great significance to the prognosis, namely, prednisone 60mg/m2 1 week/day, and immature cells in peripheral blood ≥ 1× 109/L were high-risk factors, which was called prednisone induction test. At the same time, extramedullary infiltration was also found [for example, to diagnose central nervous system leukemia, leukemia cells need to be found in cerebrospinal fluid.
Another therapeutic response is to reexamine the bone marrow on day 19 after induction remission treatment. There may be three cases: ① Bone marrow significantly inhibits primary lymphoma+juvenile lymphoma by 25%, of which 1 has a good prognosis, and the third case has a poor prognosis. If there are still immature cells in peripheral blood at this time, it is also an indication of poor prognosis, which has nothing to do with the number of primary lymph and juvenile lymph in bone marrow.
(1) The most important treatment-related factor in induced remission therapy is the time of CR. Studies have shown that the number of immature cells in peripheral blood is reduced by half 5 days after chemotherapy or the number of primitive lymphocytes in bone marrow is reduced to less than 5% within 2 weeks after chemotherapy, and the prognosis is good. In the induced remission treatment of ALL in children in Beijing Children's Hospital, more than 90% of children's bone marrow reached CR within 2 weeks after strong chemotherapy, which greatly improved the survival time and made the high-risk five-year EFS reach 74%.
(2) It is also important to treat minimal residual disease (MRD) after the bone marrow reaches CR. In the treatment of ALL in children, it is necessary to adhere to long-term maintenance treatment and preventive treatment in shelters, and the course of treatment takes 2.5 ~ 3 years.
(3) Drug resistance of primary and secondary leukemia is an important reason for the failure of chemotherapy. In recent ten years, the clinical research of multidrug resistance (MDR 1) has made rapid progress, which is expected to reverse MDR 1 gene and overcome the unfavorable factors in this treatment.
There are many factors affecting the prognosis of ALL in children, which are interrelated and influence each other. The human body is a very complicated body. Methods such as single factor or multi-factor regression analysis have limitations. Among the risk factors before treatment, the abnormal white blood cell count of age has a great influence on the prognosis, but the risk factors of ALL can be significantly reduced by adjusting the treatment plan, intensive chemotherapy, individualized treatment or bone marrow transplantation.
3. Prognostic indicators are based on the understanding of the influence of ALL for many years. 1998 The prognostic indicators set by the Pediatric Branch of Shandong Rongcheng Chinese Medical Association are:
(1) 1 infant leukemia under one year old
(2) There is central nervous system leukemia or testicular leukemia (or other clear clinical manifestations of extramedullary leukemia) at the time of diagnosis.
(3) Chromosome karyotype is T (4; 1 1) or t (9; 22) Abnormal
(4) Low diploid with less than 45 chromosomes.
(5) White blood cells in peripheral blood >: 50× 109/L at the time of diagnosis (or the history of white blood cells in peripheral blood ≥50× 109/L)
(6) On the 8th day of prednisone induction test, immature cells in peripheral blood were ≥/kloc-0 /×109/L.
(7) Patients who failed to get complete remission after 6 weeks of Sr-All induction chemotherapy.
Those who have any one or more of the above items are diagnosed as high-risk lymphoma, do not have any of the above items or have t (12; 2 1) B-ALL is a standard emergency shower.
Prevention: 1. Avoid contact with harmful factors, avoid contact with harmful chemicals, ionizing radiation and other factors that cause leukemia, and strengthen various protective measures when contacting poisons or radioactive substances; Avoid environmental pollution, especially indoor environmental pollution; Pay attention to rational drug use and use cytotoxic drugs with caution.
2 vigorously carry out the prevention and treatment of various infectious diseases, especially viral infectious diseases. Do a good job in vaccination.
3. Do a good job in eugenics and prevent some congenital diseases, such as 2 1- trisomy and Fanconi anemia. Strengthen physical exercise, pay attention to food hygiene, maintain a happy mood, combine work and rest, and enhance physical resistance.
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