Protracted and Variable Latency of Acute Lymphoblastic Leukemia AfterTEL-AML1 Gene Fusion In Utero: Presented at the American Society of Hematology Meeting, held in …

JL Wiemels, AM Ford, ER Van Wering… - Blood, The Journal …, 1999 - ashpublications.org
JL Wiemels, AM Ford, ER Van Wering, A Postma, M Greaves
Blood, The Journal of the American Society of Hematology, 1999ashpublications.org
We report a pair of identical twins with concordant acute lymphoblastic leukemia (ALL).
Unusually, their diagnoses were spaced 9 years apart at ages 5 and 14. Leukemic cells in
both twins had a TEL-AML1 rearrangement, which was characterized at the DNA level by an
adaptation of a long distance polymerase chain reaction (PCR) method. The genomic fusion
sequence was identical in the two leukemias, indicative of a single cell origin in one fetus, in
utero. At the time twin 1 was diagnosed (aged 5 years), the bone marrow of twin 2 was …
We report a pair of identical twins with concordant acute lymphoblastic leukemia (ALL). Unusually, their diagnoses were spaced 9 years apart at ages 5 and 14. Leukemic cells in both twins had aTEL-AML1 rearrangement, which was characterized at the DNA level by an adaptation of a long distance polymerase chain reaction (PCR) method. The genomic fusion sequence was identical in the two leukemias, indicative of a single cell origin in one fetus, in utero. At the time twin 1 was diagnosed (aged 5 years), the bone marrow of twin 2 was hematologically normal. However, retrospective scrutiny of the DNA from an archived slide with clonotypic TEL-AML1 primers showed that the presumptive preleukemic clone was present and disseminated 9 years before a clinical diagnosis. These data provide novel insight into the natural history of childhood leukemia and suggest that consequent to a prenatal initiation of a leukemic clone, most probably by TEL-AML fusion itself, the latency of ALL can be both extremely variable and protracted. This, in turn, is likely to reflect the timing of critical secondary events.
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