Wilms' Tumor (WT1) Gene Mutations Occur Mainly in Acute Myeloid Leukemia and May Confer Drug Resistance

摘要: In a previous study of acute leukemia, we have shown that WT1 gene mutations occur in both myeloid and biphenotypic subtypes, where they are associated with refractoriness to standard induction chemotherapy. We now extended this total 67 cases (34 leukemia [AML], 23 lymphoblastic [ALL], 10 undifferentiated [AUL]/biphenotypic) find 14% AML 20% but rare ALL (one case). contrast the findings Wilms' tumor, usually behave according Knudson's two hit model for tumor suppressor genes, seven eight leukemia-associated heterozygous, implying dominant or dominant-negative mode action hematopoietic cells. AML, presence mutation is failure achieve complete remission lower survival rate. These data (1) confirm underlie similar proportion seen tumors (2) show first time can contribute leukemogenesis lymphoid as well origin, suggesting its normal role hematopoiesis lies at very early progenitor stage. The relationship chemoresistance merits further investigation.