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 Blood, 30 July 2009, Vol. 114, No. 5, pp. 957-964.
Prepublished online as a Blood First Edition Paper on May 4, 2009; DOI 10.1182/blood-2009-03-210591.

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CLINICAL TRIALS AND OBSERVATIONS

De novo deletion 17p13.1 chronic lymphocytic leukemia shows significant clinical heterogeneity: the M. D. Anderson and Mayo Clinic experience

Constantine S. Tam1,2, Tait D. Shanafelt3, William G. Wierda1, Lynne V. Abruzzo4, Daniel L. Van Dyke5, Susan O'Brien1, Alessandra Ferrajoli1, Susan A. Lerner1, Alice Lynn1, Neil E. Kay3, and Michael J. Keating1

1 Leukemia Department, University of Texas M. D. Anderson Cancer Center, Houston; 2 Hematology Department, St Vincent's Hospital, Melbourne, Australia; 3 Hematology Department, Mayo Clinic, Rochester, MN; 4 Hematopathology Department, University of Texas M. D. Anderson Cancer Center, Houston; and 5 Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN

To determine the clinical fate of patients with de novo deletion 17p13.1 (17p–) chronic lymphocytic leukemia (CLL), we retrospectively studied the outcome of 99 treatment-naive 17p– CLL patients from the M. D. Anderson Cancer Center (n = 64) and the Mayo Clinic (n = 35). Among 67 asymptomatic patients followed for progression, 53% developed CLL requiring treatment over 3 years. Patients who had not progressed by 18 months subsequently had stable disease, with 3 of 19 patients progressing after follow-up of up to 70 months. Risk factors for progressive disease were Rai stage of 1 or higher and unmutated immunoglobulin variable region heavy chain (IgVH). The overall survival rate was 65% at 3 years. Rai stage 1 or higher, unmutated IgVH, and 17p– in 25% or more of nuclei were adverse factors for survival. The 3-year survival rates of patients with 1 or fewer, 2, and 3 of these factors were 95%, 74%, and 22%, respectively (P < .001). Response rates to therapy with rituximab (n = 6); purine analogues and rituximab (n = 25); and purine analogues, rituximab, and alemtuzumab (n = 16) combinations were 50%, 72%, and 81%, respectively. Patients with 17p– CLL exhibit clinical heterogeneity, with some patients experiencing an indolent course. Survival can be predicted using clinical and biologic characteristics.


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