Unusual immunoglobulin and T-cell receptor gene rearrangement patterns in acute lymphoblastic leukemias

T. Szczepański*, M. J. Pongers-Willemse, A. W. Langerak, J. J.M. Van Dongen

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

40 Citations (Scopus)

Abstract

Immunoglobulin (Ig) and T-cell receptor (TCR) genes are rearranged in virtually all acute lymphoblastic leukemia (ALL) cases. However, the recombination patterns display several unusual features as compared to normal lymphoid counterparts. Cross-lineage gene rearrangements occur in more than 90% of precursor-B-ALL and in ~20% of T-ALL, whereas they are rare in normal lymphocytes. Approximately 25-30% of the Ig and TCR gene rearrangements at diagnosis are oligoclonal, and can undergo continuing or secondary recombination events during the disease course. Based on our extensive molecular studies we hypothesize that the unsual Ig and TCR gene rearrangements in ALL occur as an early postoncogenic event resulting from the continuing V(D)J recombinase activity on accessible gene loci. This hypothesis is on the one hand supported by the virtual absence of cross- lineage gene rearrangements in normal lymphocytes and mature lymphoid malignancies and on the other hand by the presence of oligoclonality and secondary Ig and TCR gene rearrangements in ALL.

Original languageEnglish
Pages (from-to)205-215
Number of pages11
JournalCurrent Topics in Microbiology and Immunology
Volume246
DOIs
Publication statusPublished - 1999

Bibliographical note

© Springer-Verlag Berlin Heidelberg 1999

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