Abstract

<div>Abstract<p><b>Purpose:</b> Few biological prognosticators are useful for prediction of Richter syndrome (RS), representing the transformation of chronic lymphocytic leukemia (CLL) to aggressive lymphoma. Stereotyped B-cell receptors (BCR) may have prognostic effect in CLL progression. We tested the prognostic effect of stereotyped BCR for predicting RS transformation.</p><p><b>Experimental Design:</b> The prevalence of stereotyped BCR was compared in RS (<i>n</i> = 69) versus nontransformed CLL (<i>n</i> = 714) by a case-control analysis. Subsequently, the effect of stereotyped BCR at CLL diagnosis on risk of RS transformation was actuarially assessed in a consecutive CLL series (<i>n</i> = 753).</p><p><b>Results:</b> RS (<i>n</i> = 69) displayed a higher prevalence of stereotyped BCR (<i>P</i> < 0.001) compared with nontransformed CLL. The actuarial risk of RS transformation was significantly higher in CLL carrying stereotyped BCR (<i>P</i> < 0.001). Among BCR subsets most represented in CLL, subset 8 using <i>IGHV4-39/IGHD6-13/IGHJ5</i> carried the highest risk of RS transformation [hazard ratio (HR), 24.50; <i>P</i> < 0.001]. Multivariate analysis selected stereotyped BCR (HR, 3.33; <i>P</i> = 0.001) and <i>IGHV4-39</i> usage (HR, 4.03; <i>P</i> = 0.004) as independent predictors of RS transformation. The combination of <i>IGHV4-39</i> usage and stereotyped BCR in the same patient identified CLL with a very high risk of RS transformation (5-year risk, 68.7%). The risk carried by stereotyped BCR and <i>IGHV4-39</i> usage was specific for RS transformation and had no effect on CLL progression without transformation.</p><p><b>Conclusions:</b> Analysis of BCR features may help identify CLL patients at risk of RS. A close monitoring and a careful biopsy policy may help early recognition of RS in CLL patients using stereotyped BCR, particularly if combined with <i>IGHV4-39</i>.</p></div>

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