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ZAP-70 Compared with Immunoglobulin Heavy-Chain Gene Mutation Status as a Predictor of Disease Progression in Chronic Lymphocytic Leukemia

Rassenti, Laura Z., Huynh, Lang, Toy, Tracy L., Chen, Liguang, Keating, Michael J., Gribben, John G., Neuberg, Donna S., Flinn, Ian W., Rai, Kanti R., Byrd, John C., Kay, Neil E., Greaves, Andrew, Weiss, Arthur, Kipps, Thomas J.·2004·New England Journal of Medicine

Abstract

Background The course of chronic lymphocytic leukemia (CLL) is variable. In aggressive disease, the CLL cells usually express an unmutated immunoglobulin heavy-chain variable-region gene (IgV(H)) and the 70-kD zeta-associated protein (ZAP-70), whereas in indolent disease, the CLL cells usually express mutated IgV(H) but lack expression of ZAP-70. Methods We evaluated the CLL B cells from 307 patients with CLL for ZAP-70 and mutations in the rearranged IgV(H) gene. We then investigated the association between the results and the time from diagnosis to initial therapy. Results We found that ZAP-70 was expressed above a defined threshold level in 117 of the 164 patients with an unmutated IgV(H) gene (71 percent), but in only 24 of the 143 patients with a mutated IgV(H) gene (17 percent, P Conclusions Although the presence of an unmutated IgV(H) gene is strongly associated with the expression of ZAP-70, ZAP-70 is a stronger predictor of the need for treatment in B-cell CLL.

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Metadata

Authors
Rassenti, L. Z., Huynh, L., Toy, T. L., Chen, L., Keating, M. J., Gribben, J. G., Neuberg, D. S., Flinn, I. W., Rai, K. R., Byrd, J. C., Kay, N. E., Greaves, A., Weiss, A., & Kipps, T. J.
Venue
New England Journal of Medicine
Published
2004-08-26
Volume
351
Issue
9
Pages
893-901
ISSN
  • 0028-4793
  • 1533-4406
Cited by
745
References
0
Language
en

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CoChat. (2026). Citation check: ZAP-70 Compared with Immunoglobulin Heavy-Chain Gene Mutation Status as a Predictor of Disease Progression in Chronic Lymphocytic Leukemia. https://app.cochat.ai/papers/10.1056/nejmoa040857
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Rassenti, L. Z., Huynh, L., Toy, T. L., Chen, L., Keating, M. J., Gribben, J. G., Neuberg, D. S., Flinn, I. W., Rai, K. R., Byrd, J. C., Kay, N. E., Greaves, A., Weiss, A., & Kipps, T. J. (2004). ZAP-70 Compared with Immunoglobulin Heavy-Chain Gene Mutation Status as a Predictor of Disease Progression in Chronic Lymphocytic Leukemia. New England Journal of Medicine. https://doi.org/10.1056/nejmoa040857