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.

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

Visentin, Andrea
Membro del Collaboration Group
;
Imbergamo, Silvia
Membro del Collaboration Group
;
Frezzato, Federica
Membro del Collaboration Group
;
Pizzi, Marco
Membro del Collaboration Group
;
Bertorelle, Roberta
Membro del Collaboration Group
;
Scomazzon, Edoardo
Membro del Collaboration Group
;
Berno, Tamara
Membro del Collaboration Group
;
Riva, Marcello
Membro del Collaboration Group
;
Piva, Elisa
Membro del Collaboration Group
;
Facco, Monica
Membro del Collaboration Group
;
Piazza, Francesco
Membro del Collaboration Group
;
Semenzato, Gianpietro;Trentin, Livio
Membro del Collaboration Group
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.
2017
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/3253750
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