Factors predicting survival in chronic lymphocytic leukemia patients developing Richter syndrome transformation into Hodgkin lymphoma

Francesca Romana Mauro, Piero Galieni, Alessandra Tedeschi, Luca Laurenti, Giovanni Del Poeta, Gianluigi Reda, Marina Motta, Alessandro Gozzetti, Roberta Murru, Maria Denise Caputo, Melissa Campanelli, Anna Maria Frustaci, Idanna Innocenti, Sara Raponi, Anna Guarini, Fortunato Morabito, Robin Foà, Massimo Gentile

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Abstract

We hereby report the clinical and biologic features of 33 of 4680 (0.7%) patients with chronic lymphocytic leukemia (CLL), managed at 10 Italian centers, who developed Hodgkin lymphoma (HL), a rare variant of Richter syndrome. The median age at CLL and at HL diagnosis were 61 years (range 41-80) and 70 years (range 46-82), respectively, with a median interval from CLL to the diagnosis of HL of 90 months (range 0-258). In 3 cases, CLL and HL were diagnosed simultaneously. Hl was characterized by advanced stage in 79% of cases, International Prognostic Score (IPS) ≥4 in 50%, extranodal involvement in 39%, B symptoms in 70%. Prior treatment for CLL had been received by 82% of patients and included fludarabine in 67%. Coexistence of CLL and HL was detected in the same bioptic tissue in 87% of cases. The most common administered treatment was the ABVD regimen given to 22 patients (66.6%). The complete response (CR) rate after ABVD was 68%, and was influenced by the IPS (P =.03) and interval from the last CLL treatment (P =.057). Survival from HL was also influenced by the IPS (P =.006) and time from the last CLL treatment (P =.047). The achievement of CR with ABVD was the only significant and independent factor predicting survival (P =.037). Taken together, our results show that the IPS and the interval from the prior CLL treatment influence the likelihood of achieving CR after ABVD, which is the most important factor predicting survival of patients with CLL developing HL.

Original languageEnglish
Pages (from-to)529-535
Number of pages7
JournalAmerican Journal of Hematology
Volume92
Issue number6
DOIs
Publication statusPublished - Jun 1 2017

ASJC Scopus subject areas

  • Hematology

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    Mauro, F. R., Galieni, P., Tedeschi, A., Laurenti, L., Del Poeta, G., Reda, G., Motta, M., Gozzetti, A., Murru, R., Caputo, M. D., Campanelli, M., Frustaci, A. M., Innocenti, I., Raponi, S., Guarini, A., Morabito, F., Foà, R., & Gentile, M. (2017). Factors predicting survival in chronic lymphocytic leukemia patients developing Richter syndrome transformation into Hodgkin lymphoma. American Journal of Hematology, 92(6), 529-535. https://doi.org/10.1002/ajh.24714