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Am J Hematol. 2017 Jun;92(6):529-535. doi: 10.1002/ajh.24714. Epub 2017 Apr 18.

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

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Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
U.O.C. Ematologia e Trapianto di Cellule Staminali Emopoietiche, Ospedale Mazzoni, Ascoli Piceno.
Department of Hematology, Niguarda Cancer Center, Milan.
Department of Hematology, Catholic University, Rome.
Hematology, Dipartimento di Biomedicina e Prevenzione, Università degli Studi di Roma Tor Vergata, Rome.
Oncohematology Department, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan.
Department of Hematology, ASST Spedali Civili, Brescia.
Division of Hematology, University of Siena, Siena.
UO Ematologia e CTMO Ospedale Oncologico A. Businco, Cagliari.
Hematology Section, Cosenza Hospital, Cosenza, Italy.


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.

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