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SIMPLISTIC CLASSIFICATION OF LYMPHOID LEUKEMIAS. ACUTE LYMPHOID LEUKEMIA PRECURSOR B-ALL Specific molecular abnormalities PRECURSOR T-ALL Specific molecular abnormalities BURKITT’S LEUKEMIA CHRONIC LYMPHOID LEUKEMIAS B-CLL Other.
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SIMPLISTIC CLASSIFICATION OF LYMPHOID LEUKEMIAS • ACUTE LYMPHOID LEUKEMIA • PRECURSOR B-ALL • Specific molecular abnormalities • PRECURSOR T-ALL • Specific molecular abnormalities • BURKITT’S LEUKEMIA • CHRONIC LYMPHOID LEUKEMIAS • B-CLL • Other
FOR ALL INTENTS AND PURPOSES B-CLL DOES NOT OCCUR IN CHILDREN Of about 5000 childhood leukemias sent to my reference lab I have seen 2 cases of B-CLL Other chronic lymphoproliferative disorders are rare in children and are probably best discussed under lymphomas
Precursor B-ALL t(9;22) (BCR-ABL) t(v;11) MLL t(1;19) (E2A-PBX1) t(12;21) (TEL-AML1) Hyperdiploidy ?Hypodiploidy Precursor T-ALL SCL1(TAL) HOX11 LMO1/2(RBTN1/2) LYL1 ?Primitive T-ALL ACUTE LYMPHOID LEUKEMIAImportant entities
DIFFERENCES BETWEEN ADULT AND CHILDHOOD ALLT-ALL • No good data on frequency differences among genetically-defined groups • No good data that genetically-defined groups fare differently • Thus, for current purposes, T-ALL in adults and children are likely the same disease • Frequency of T-ALL higher in adults than children
DIFFERENCES BETWEEN GENETICS IN ADULT AND CHILDHOOD ALLPRECURSOR B-ALL
GENETICS AND PROGNOSIS IN CHILDHOOD ALL • GOOD PROGNOSIS • Hyperdiploidy • TEL-AML1 • POOR PROGNOSIS • BCR-ABL • MLL • INTERMEDIATE PROGNOSIS • E2A-PBX1(if treated appropriately) • Other
CHILDHOOD VS ADULT ALLSUMMARY • Good prognosis genetic lesions in children rare in adults and should be considered distinct diseases • There are no significant differences between adult or childhood Burkitt’s leukemia, T-ALL, or leukemias with bcr-abl or MLL abnormalities, so that any protocols targeting these diseases should include both adults and children • Remaining 40% of childhood precursor B-ALL is not noticeably biologically different from adults, but still probably fare better