title: Factors predicting survival in chronic lymphocytic leukemia patients developing Richter syndrome transformation into Hodgkin lymphoma.
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.
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Francesca Romana
authors.identifier: http://orcid.org/0000-0003-2425-9474
authors.initials: FR
authors.lastname: Mauro
authors.affiliation: U.O.C. Ematologia e Trapianto di Cellule Staminali Emopoietiche, Ospedale Mazzoni, Ascoli Piceno.
authors.forename: Piero
authors.initials: P
authors.lastname: Galieni
authors.affiliation: Department of Hematology, Niguarda Cancer Center, Milan.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Tedeschi
authors.affiliation: Department of Hematology, Catholic University, Rome.
authors.forename: Luca
authors.initials: L
authors.lastname: Laurenti
authors.affiliation: Hematology, Dipartimento di Biomedicina e Prevenzione, Università degli Studi di Roma Tor Vergata, Rome.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Del Poeta
authors.affiliation: Oncohematology Department, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan.
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Reda
authors.affiliation: Department of Hematology, ASST Spedali Civili, Brescia.
authors.forename: Marina
authors.initials: M
authors.lastname: Motta
authors.affiliation: Division of Hematology, University of Siena, Siena.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gozzetti
authors.affiliation: UO Ematologia e CTMO Ospedale Oncologico A. Businco, Cagliari.
authors.forename: Roberta
authors.initials: R
authors.lastname: Murru
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Maria Denise
authors.initials: MD
authors.lastname: Caputo
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Melissa
authors.initials: M
authors.lastname: Campanelli
authors.affiliation: Department of Hematology, Niguarda Cancer Center, Milan.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Frustaci
authors.affiliation: Department of Hematology, Catholic University, Rome.
authors.forename: Idanna
authors.initials: I
authors.lastname: Innocenti
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Sara
authors.initials: S
authors.lastname: Raponi
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Anna
authors.initials: A
authors.lastname: Guarini
authors.affiliation: Hematology Section, Cosenza Hospital, Cosenza, Italy.
authors.forename: Fortunato
authors.initials: F
authors.lastname: Morabito
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Policlinico Umberto I, Rome.
authors.forename: Robin
authors.initials: R
authors.lastname: Foà
authors.affiliation: Hematology Section, Cosenza Hospital, Cosenza, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Gentile
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001761:Bleomycin / Q000627:therapeutic use; D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D003606:Dacarbazine / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006689:Hodgkin Disease / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009154:Mutation; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use
chemical_list: D007143:Immunoglobulin Heavy Chains; D001761:Bleomycin; D014747:Vinblastine; D003606:Dacarbazine; D004317:Doxorubicin