Rossi, Davide, Spina, Valeria, Cerri, Michaela, Rasi, Silvia, Deambrogi, Clara, De Paoli, Lorenzo, Laurenti, Luca, Maffei, Rossana, Forconi, Francesco, Bertoni, Francesco, Zucca, Emanuele, Agostinelli, Claudio, Cabras, Antonello, Lucioni, Marco, Martini, Maurizio, Magni, Michele, Deaglio, Silvia, Ladetto, Marco, Nomdedeu, Josep F, Besson, Caroline, Ramponi, Antonio, Canzonieri, Vincenzo, Paulli, Marco, Marasca, Roberto, Larocca, Luigi M, Carbone, Antonino, Pileri, Stefano A, Gattei, Valter and Gaidano, Gianluca (2009) Stereotyped B-cell receptor is an independent risk factor of chronic lymphocytic leukemia transformation to Richter syndrome. Clinical Cancer Research, 15 (13), 4415-4422. (doi:10.1158/1078-0432.CCR-08-3266). (PMID:19509140)
Abstract
PURPOSE: 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.
EXPERIMENTAL DESIGN: The prevalence of stereotyped BCR was compared in RS (n = 69) versus nontransformed CLL (n = 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 (n = 753).
RESULTS: RS (n = 69) displayed a higher prevalence of stereotyped BCR (P < 0.001) compared with nontransformed CLL. The actuarial risk of RS transformation was significantly higher in CLL carrying stereotyped BCR (P < 0.001). Among BCR subsets most represented in CLL, subset 8 using IGHV4-39/IGHD6-13/IGHJ5 carried the highest risk of RS transformation [hazard ratio (HR), 24.50; P < 0.001]. Multivariate analysis selected stereotyped BCR (HR, 3.33; P = 0.001) and IGHV4-39 usage (HR, 4.03; P = 0.004) as independent predictors of RS transformation. The combination of IGHV4-39 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 IGHV4-39 usage was specific for RS transformation and had no effect on CLL progression without transformation.
CONCLUSIONS: 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 IGHV4-39.
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