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. 2009 Jun 25;113(26):6691-4.
doi: 10.1182/blood-2009-01-202259. Epub 2009 May 1.

Discordance of MLL-rearranged (MLL-R) infant acute lymphoblastic leukemia in monozygotic twins with spontaneous clearance of preleukemic clone in unaffected twin

Meredith K Chuk  1 , Emily McIntyre, Donald Small, Patrick Brown
Affiliations

Discordance of MLL-rearranged (MLL-R) infant acute lymphoblastic leukemia in monozygotic twins with spontaneous clearance of preleukemic clone in unaffected twin

Meredith K Chuk et al. Blood. .

Abstract

Concordance of MLL-rearranged acute leukemia in infant monozygotic twins is thought to be 100% with a very short latency period, suggesting that either the MLL fusion itself is sufficient to cause leukemia or that it promotes the rapid acquisition of additional oncogenic events that result in overt disease. We report the first case of discordance in an infant monozygotic twin pair. Twin A presented at age 9 months with MLL-ENL(+) acute lymphoblastic leukemia and twin B remains healthy 3 years later. The presence and eventual clearance of a clonal population of MLL-ENL(+) cells was shown in the bone marrow and peripheral blood of twin B. Clearance of this clone was temporally associated with viral-induced cytopenias, suggesting an immune-mediated clearance of the clone before the development of leukemia. Thus, concordance of MLL-rearranged acute leukemia in infant monozygotic twins is not universal. The implications of this case for MLL-rearranged leukemogenesis are discussed.

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Figures

Figure 1
Figure 1
Twin B's ANC, platelet count, and MLL-ENL RT-PCR status over time from twin A's diagnosis. ANC indicates absolute neutrophil count; PB, peripheral blood; and BM, bone marrow.
Figure 2
Figure 2
Laboratory results. (A) MLL-ENL RT-PCR for twins A and B with actin control. (B) Summary of MLL-ENL RT-PCR and MLL FISH results for twins A and B; BM indicates bone marrow; PB, peripheral blood; n/a, not applicable; nd, not done. (C) Sequencing results (wt MLL and ENL sequences included as reference).
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References

    1. Greaves MF, Maia AT, Wiemels JL, Ford AM. Leukemia in twins: lessons in natural history. Blood. 2003;102:2321–2333. - PubMed
    1. Ford AM, Ridge SA, Cabrera ME, et al. In utero rearrangements in the trithorax-related oncogene in infant leukaemias. Nature. 1993;363:358–360. - PubMed
    1. Gill Super HJ, Rothberg PG, Kobayashi H, Freeman AI, Diaz MO, Rowley JD. Clonal, nonconstitutional rearrangements of the MLL gene in infant twins with acute lymphoblastic leukemia: in utero chromosome rearrangement of 11q23. Blood. 1994;83:641–644. - PubMed
    1. Jansen MW, Corral L, van der Velden VH, et al. Immunobiological diversity in infant acute lymphoblastic leukemia is related to the occurrence and type of MLL gene rearrangement. Leukemia. 2007;21:633–641. - PubMed
    1. Mullighan CG, Goorha S, Radtke I, et al. Genome-wide analysis of genetic alterations in acute lymphoblastic leukaemia. Nature. 2007;446:758–764. - PubMed

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