T1517Pmlrara and FLT3 ITD

PML-RARA contributes to the development of APL. However, incomplete penetrance and long latency observed in PML-RARA transgenic mice suggest that additional events are required for complete leukemic transformation. The observation that FLT3 mutations are found in about one-third of patients with t(15;17) has led to the hypothesis that the primary translocation event might impair differentiation and a second hit, such as the FLT3 ITD, confers the proliferation stimulus for the leukemic cells.67 Indeed, experimental data demonstrate that PML-RARA and FLT3 ITD cooperate and that the two events lead to an ATRA-responsive APL-like disease with a short latency and 100% penetrance.68

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