Bendamustine plus rituximab is an effective first-line treatment in hairy cell leukemia variant: a report of three cases

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// Andrea Visentin 1, 2 , Silvia Imbergamo 1 , Federica Frezzato 1, 2 , Marco Pizzi 3 , Roberta Bertorelle 4 , Edoardo Scomazzon 1 , Tamara Berno 1 , Marcello Riva 1 , Elisa Piva 5 , Monica Facco 1, 2 , Francesco Piazza 1, 2 , Gianpietro Semenzato 1, 2 and Livio Trentin 1, 2 1 Hematology and Clinical Immunology Unit, Department of Medicine, University of Padua, Padua, Italy 2 Venetian Institute of Molecular Medicine, Centro di Eccellenza per la Ricerca Biomedica Avanzata, Padua, Italy 3 General Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Padua, Italy 4 Immunology and Molecular Diagnostic Oncology Unit, Veneto Institute of Oncology IOV-IRCSS, Padua, Italy 5 Unity of Laboratory Medicine, University of Padua, Padua, Italy Correspondence to: Livio Trentin, email: livio.trentin@unipd.it Keywords: HCL, HCL variant, bendamustine, BR, treatment naive Received: April 21, 2017      Accepted: September 15, 2017      Published: September 28, 2017 ABSTRACT Hairy cell leukemia variant (HCLv) is a chronic lymphoproliferative disorder classified as a provisional entity in the 2016 WHO Classification of Lymphoid Tumors. HCLv is characterized by unfavorable prognosis, low complete remission rates and limited disease control following classical hairy cell leukemia-based regimens. In this study, we report 3 cases of elderly patients with treatment-naive, TP53 un-mutated HCLv, who were effectively treated with four cycles of bendamustine plus rituximab. The regimen was completed in all the patients with acceptable toxicity. All patients achieved a complete clinical response with no evidence of residual disease at bone marrow biopsy and flow-cytometry examination. After a median follow-up of 19 months, the 3 subjects are still in complete remission. In this work, bendamustine plus rituximab proved to be an effective and feasible first-line treatment strategy for elderly patients with TP53 un-mutated HCLv.