Please use this identifier to cite or link to this item: http://hdl.handle.net/11434/2031
Title: Survival outcomes for plasmablastic lymphoma: an international, multicentre study by the Australasian Lymphoma Alliance.
Epworth Authors: Yannakou, Costas
Other Authors: Di Ciaccio, Pietro
Polizzotto, Mark
Cwynarski, Kate
Burton, Cathy
Jiamsakul, Awachana
Bower, Mark
Kuruvilla, John
Montoto, Silvia
McKay, Pam
Osborne, Wendy
Milliken, Sam
Linton, Kim
Manos, Kate
Kassam, Shireen
Doo, Nicole
Watson, Ann-Marie
Fedele, Pasquale
Hunt, Stewart
Renshaw, Hanna
Thakrar, Nisha
Smith, Alexandra
Painter, Daniel
Maxwell, Alice
Liu, Qin
Dhairyawan, Rageshri
Ferguson, Graeme
Pickard, Keir
Hamad, Nada
Keywords: Plasmablastic Lymphoma (PBL)
Immunodeficiency
Oncogenic Impact of EBV
Dysregulated Immune Surveillance
Acquired Abnormalities
Plasma Cell Markers
Classical B Cell Markers
Median Survival
Cox Regression
Overall Survival (OS)
Immunosuppressive Risk factors
Multinational Retrospective Cohort
Early-Stage Disease
Baseline Bone Marrow Involvement
Inferior OS
Baseline Bone Marrow Biopsy
PET Staging
PET Imaging
Novel Agents in PBL
Australasian Lymphoma Alliance
HIV Infection
Solid Organ Transplantation
Understanding the Pathogenesis of PBL
CD138
CD38
CD30
CD20
CD19
PAX5
CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone)-Based Chemotherapy
Epworth Centre for Immunotherapies and Snowdome Laboratories
Molecular Oncology and Cancer Immunology
Cancer Services Clinical Institute, Epworth HealthCare, Victoria, Australia
Issue Date: Nov-2020
Publisher: American Society of Hematology (ASH)
Citation: Blood (2020) 136 (Supplement 1): 1–2.
Abstract: Plasmablastic lymphoma (PBL) is a rare, aggressive large cell lymphoma, first described in 1997. PBL is strongly associated with immunodeficient states, such as HIV infection and solid organ transplantation, but up to one third of cases are reported to occur in immunocompetent patients. The pathogenesis of PBL is incompletely understood, though the oncogenic impact of EBV, in particular in the context of dysregulated immune surveillance, together with acquired abnormalities in the MYC pathway appear to play key roles in many cases. Plasma cell markers such as CD138 and CD38 are typically positive, as well as CD30 in a significant subset. Classical B cell markers such as CD20, CD19 and PAX5 are typically absent. The literature on clinical outcomes in PBL is generally limited to small, single-centre case series. Reports describe an aggressive disease of poor prognosis, with median survival of 8 to 15 months, with one series reporting a longer median survival of 32 months. We retrospectively identified patients diagnosed with PBL between 1999 and 2019 from 16 sites across Australia, the United Kingdom and Canada. Patients aged ≥18 years with confirmed tissue diagnosis of PBL at their local treating centre were included. Factors associated with overall survival (OS) were analysed using Cox regression, stratified by site to account for heterogeneity across sites. Risk time for mortality began on the date of diagnosis and ended on the date of death. Patients who were alive, lost to follow-up or transferred to another centre for care, were censored on the date of last follow-up. Risk factors analysed included age, year of diagnosis, HIV status, MYC rearrangement status, CD30 status, lactate dehydrogenase level, disease stage by Lugano consensus criteria, and bone marrow involvement. We report a multinational retrospective cohort of patients diagnosed with PBL and to our knowledge the largest single series of PBL to date. OS was longer than previously published data, particularly in patients with early-stage disease. However, patients with stage IV disease and baseline bone marrow involvement had inferior OS. HIV infection did not affect outcome. These findings suggest that baseline bone marrow biopsy and PET staging are useful prognostic tools. There is also an ongoing need for the evaluation of the predictive value of PET imaging and novel agents in PBL, especially in higher-risk disease.
URI: http://hdl.handle.net/11434/2031
DOI: 10.1182/blood-2020-134972
ISSN: 0006-4971
1528-0020
Journal Title: Blood
Type: Journal Article
Affiliated Organisations: Department of Haematology and Bone Marrow Transplantation, St Vincent's Hospital, Sydney, Australia
University of New South Wales, Sydney, Australia
The Kirby Institute, University of New South Wales, Sydney, NSW, Australia
Department of Haematology, University College Hospital, London, United Kingdom
Department of Haematology, St James University Hospital, Leeds, United Kingdom
National Centre for HIV Malignancy, Chelsea & Westminster Hospital, London, United Kingdom
The Princess Margaret Hospital, Toronto, ON, Canada
Department of Haemato-oncology, St Bartholomew's Hospital, Barts Health NHS Trust, London, United Kingdom
Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom
Newcastle upon Tyne NHS Foundation Trust, Newcastle-upon-Tyne, United Kingdom
The Christie, Manchester, United Kingdom
University of Manchester, Manchester, United Kingdom
Manchester Academic Health Science Centre, Manchester, United Kingdom
Department of Haematology, Austin Health, Melbourne, Australia
King's College Hospital, London, United Kingdom
Concord Repatriation General Hospital, Sydney, NSW, Australia
University of Sydney, Sydney, NSW, Australia
Department of Haematology, Liverpool Hospital, Sydney, NSW, Australia
School of Clinical Sciences at Monash Health, Monash University, Clayton, Australia
Haematology Department, Monash Health, Clayton, Australia
Department of Molecular Oncology and Cancer Immunology, Epworth HealthCare, East Melbourne, VIC, Australia
Department of Haematology, Gold Coast University Hospital, Gold Coast, QLD, Australia
University College Hospital, London, United Kingdom
Epidemiology and Cancer Statistics Group, Department of Heath Sciences, University of York, York, United Kingdom
Epidemiology and Cancer Statistics Group, Department of Health Sciences, University of York, York, GBR
Chelsea & Westminster Hospital, London, United Kingdom
Department of Haematology, Princess Margaret Cancer Centre, Toronto, Canada
Department of Infection and Immunity, Barts Health NHS Trust, London, United Kingdom
Department of Haematology, Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom
Faculty of Medicine, University of New South Wales, Sydney, Australia
Department of Haematology, St Vincent's Hospital, Sydney, Australia
Type of Clinical Study or Trial: Clinical Trial
Appears in Collections:Cancer Services
MOCI

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