儿童白血病英文课件教案.pptVIP

* * 白血病细胞的遗传学特征 位于11q23的MLL基因可与30多种基因发生易位，统称MLL基因重排，见于6%的急淋患儿，其中最常见的为t(4;11) (q21;q23)易位形成的融合基因MLL/AF4，绝大多数婴儿白血病表达该融合基因。最近一个大宗病例统计显示任何MLL基因重排的ALL患儿预后都不好，长期EFS只有20%~25%，尤其MLL基因重排的婴儿比1岁以上患儿预后更差。t(4;11) 阳性婴儿白血病细胞对阿糖胞苷相对敏感。 * 白血病细胞的遗传学特征 t(9;22) (q34;q11)易位形成的融合基因BCR/ABL 见于3%~5%的儿童急淋，为预后最差的一类，尤其初诊白细胞≥50?109/L、发病年龄≥10岁或强的松反应不良的患儿建议第一次缓解后即行异基因造血干细胞移植，以减少复发，提高总体生存率。 * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * THE CHILDHOOD LEUKEMIA Leukemia Leukemia is a disease characterized by proliferation of immature white cells and is the most common malignancy of childhood. Acute leukemias account for the majority (97%) of cases. * Leukemia The leukemias are classified according to the white blood cell line involved: ????Acute lymphocytic leukemia (ALL) ----- cells of lymphoid lineage Acute non-lymphocytic leukemia (ANLL) ----- cells of granulocytic or monocytic lineage. * Acute lymphocytic leukemia ALL account for 80% of childhood leukemia and has a peak in incidence between age 3 and 6 years. It is slightly more common in boys than girls. * Acute lymphocytic leukemia Children with Down syndrome, Fanconi anemia ,and ataxiatelangiectasia are at particular risk of ALL . Siblings, especially twins, of children with leukemia are approximately twice as likely to have leukemia than is the general population . . * Acute lymphocytic leukemia Some cases of childhood ALL may relate to hereditary or acquired mutation in the p53 gene. Taken in total , however , these predisposing circumstances or relationships account for only a small propotion of cases * Clinical features In most children with ALL , there is an acute onset of symptoms and signs arsing from infiltration of the bone marrow or other organs with leukemic blast cells . Most will have one or more of the following : * Clinical features ? “4 Ps” Pallor ---- anemia. Pyrexia ---- concomitant infection or the desease itself. Purpura ---- thrombocytopenia Pain ---- Bone pain (pel