Evidence map›Paper›PMID 41843467›Full record

ArticleBlood2026

Immunodeficiency-associated primary CNS lymphomas: an International Primary CNS Lymphoma Collaborative Group study.

Leon D Kaulen, Lakshmi Nayak, Philipp Karschnia, Imke Kraai, Daniela Galluzzo, Fleur A de Groot, Matthew Witterholt, Laura Donovan, Sita Bhella, Luis P Kuschel and 34 more

Abstract readMulticenter Study
In one paragraph

Article in Blood, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed.

  1. Review
  2. Review
  3. Article
  4. Review
  5. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

44 authors.

Leon D KaulenDepartment of Neurology and European Center for Neurooncology, University Hospital Heidelberg, Heidelberg University, Heidelberg, Germany.ORCID 0000-0002-5937-3376
Lakshmi NayakDepartment of Medical Oncology, Center for Neuro Oncology, Dana Farber Cancer Institute, Boston, MA.ORCID 0000-0003-2725-8717
Philipp KarschniaDivision of Neuro-Oncology, Department of Neurology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA.ORCID 0000-0002-1254-5310
Imke KraaiDepartment of Hematology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Daniela GalluzzoDepartment of Neurology, Yale School of Medicine, New Haven, CT.
Fleur A de GrootDepartment of Hematology, Leiden University Medical Center, Leiden University, Leiden, The Netherlands.ORCID 0000-0001-9940-2891
Matthew WitterholtHIV and AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.ORCID 0009-0003-0794-6157
Laura DonovanDepartment of Neuro-Oncology, Columbia University Irving Medical Center, New York, NY.
Sita BhellaDivision of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, ON, Canada.ORCID 0009-0005-9553-4340
Luis P KuschelDepartment of Neurosurgery, Ludwig Maximilian University, Munich, Germany.ORCID 0000-0001-7794-2205
Christopher P FoxDepartment of Haematology, School of Medicine, University of Nottingham, Nottingham, United Kingdom.ORCID 0000-0002-6322-9254
Sabine SeidelDepartment of Neurology, Knappschaft Kliniken University Hospital Bochum, Ruhr University Bochum, Bochum, Germany.ORCID 0000-0002-1671-3297
James PatersonDepartment of Haematology, University College London, London, United Kingdom.
Benjamin RichterDepartment of Neurology, Medical Faculty Mannheim, Mannheim Center for Translation Neuroscience, Heidelberg University, Mannheim, Germany.
Karan S DixitDivision of Neuro-Oncology, Department of Neurology, Feinberg School of Medicine Northwestern University, Chicago, IL.ORCID 0000-0003-0403-6054
Thomas ZeyenDepartment of Neurooncology, Center for Neurology and Center for Integrated Oncology, University Hospital Bonn, Bonn, Germany.
Juan Pablo AlderuccioDepartment of Medicine, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL.ORCID 0000-0002-2690-3377
Silvia MontotoDepartment of Haemato-Oncology, St. Bartholomew's Hospital, Barts Health NHS Trust, London, United Kingdom.ORCID 0000-0002-3006-9725
Outi KuittinenFaculty of Health Medicine, School of Medicine, Institute of Clinical Medicine Oncology, Kuopio University Hospital, University of Eastern Finland, Kuopio, Finland.ORCID 0000-0001-9969-0053
Benjamin-Leon TraubDepartment of Neurology and European Center for Neurooncology, University Hospital Heidelberg, Heidelberg University, Heidelberg, Germany.ORCID 0009-0008-9155-2746
Sofia DoubrovinskaiaDepartment of Neurology and European Center for Neurooncology, University Hospital Heidelberg, Heidelberg University, Heidelberg, Germany.ORCID 0000-0003-4470-8396
Hannah RohdjessDepartment of Neurology and European Center for Neurooncology, University Hospital Heidelberg, Heidelberg University, Heidelberg, Germany.ORCID 0009-0001-4985-1231
Katharina J MüllerDepartment of Neurology, Ludwig Maximilian University, Munich, Germany.ORCID 0000-0001-7098-4744
Roelien EntingDepartment of Neurology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Jeroen de BresserDepartment of Radiology, Leiden University Medical Center, Leiden, The Netherlands.ORCID 0000-0003-0759-8407
Hanne KuitunenDepartment of Oncology, Cancer Centre, Oulu University Hospital, Oulu, Finland.
Fabio M IwamotoDepartment of Neuro-Oncology, Columbia University Irving Medical Center, New York, NY.ORCID 0000-0003-1397-9927
Alvaro AlencarDepartment of Medicine, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL.ORCID 0000-0002-1720-9295
Ulrich HerrlingerDepartment of Neurooncology, Center for Neurology and Center for Integrated Oncology, University Hospital Bonn, Bonn, Germany.ORCID 0000-0002-4011-9327
Michael PlattenDepartment of Neurology, Medical Faculty Mannheim, Mannheim Center for Translation Neuroscience, Heidelberg University, Mannheim, Germany.ORCID 0000-0002-4746-887X
Kate CwynarskiDepartment of Haematology, University College London, London, United Kingdom.ORCID 0000-0001-9936-3431
Joe MendezDepartment of Neurosurgery, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT.ORCID 0000-0002-9138-8658
Patrick RothDepartment of Neurology and Brain Tumor Center, University Hospital Zurich, University of Zurich, Zurich, Switzerland.ORCID 0000-0003-3897-214X
Louisa von BaumgartenDepartment of Neurosurgery, Ludwig Maximilian University, Munich, Germany.
Anca PricaDivision of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, ON, Canada.ORCID 0000-0001-5621-9261
Govind BhagatDepartment of Pathology, Saint Louis University School of Medicine, St. Louis, MO.ORCID 0000-0001-6250-048X
Kathryn LurainHIV and AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.ORCID 0000-0002-5794-7292
David SchiffDepartment of Neurology, Division of Neuro-Oncology, University of Virginia, Charlottesville, VA.ORCID 0000-0003-1561-9220
Joost S P VermaatDepartment of Hematology, Leiden University Medical Center, Leiden University, Leiden, The Netherlands.ORCID 0000-0002-1628-6256
Joachim M BaehringDepartment of Neurology, Yale School of Medicine, New Haven, CT.ORCID 0000-0001-7755-6793
Marcel NijlandDepartment of Hematology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.ORCID 0000-0002-2740-2873
Jorg DietrichDivision of Neuro-Oncology, Department of Neurology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA.ORCID 0000-0001-8291-0136
Tracy T BatchelorDivision of Neuro-Oncology, Department of Neurology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA.ORCID 0000-0001-7568-6607
Wolfgang WickDepartment of Neurology and European Center for Neurooncology, University Hospital Heidelberg, Heidelberg University, Heidelberg, Germany.ORCID 0000-0002-6171-634X

Funding

Tumor Biology Research ProgramP30CA240139 · NCI · UNIVERSITY OF MIAMI SCHOOL OF MEDICINE · PI George S. Grills · 2019 to 2026
$24.1M
NCI National Cancer Institute - NIH National Institutes of Health P30-CA240139NCI NIH HHS P30 CA240139US Department of Defense NIH Intramural Research Program CA220385
6 · The paper itself

Abstract

abstractImmunodeficiency-associated primary central nervous system lymphoma (ID-PCNSL) represents a clinicopathologically distinct PCNSL subtype, for which large studies and prognostic models are lacking. To address this gap, the International PCNSL Collaborative Group conducted a retrospective multicenter study, integrating clinical, radiological, and pathological data from 308 ID-PCNSL cases, diagnosed at 23 participating sites in 7 countries. Preexisting immunodeficiency included administration of immunosuppressants for transplantation (41.2%) or autoimmunity (36.7%) and HIV infection (21.7%). All tumors were diffuse large B-cell lymphomas, with Epstein-Barr virus (EBV) detected in 79.2%. Immune reconstitution together with rituximab and methotrexate-based chemotherapy was associated with the highest response rates and prolonged progression-free survival, irrespective of immunodeficiency subtype and EBV status. Survival outcomes were highly variable, with a 54-month median overall survival. Multivariable Cox regression identified age (per year increment; hazard ratio [HR], 1.05 (95% confidence interval [CI], 1.02-1.07); P< .001), Karnofsky performance status (KPS) <70 (HR, 3.10; 95% CI, 1.67-5.87; P< .001), and EBV positivity (HR, 3.26; 95% CI, 1.47-7.33; P = .004) as prognostic factors for overall survival. A prognostic score was developed based on the sum of these adverse variables (age >60 years, KPS <70, EBV positivity). Stratification by this score yielded median survival times of 135, 29, and 3 months in patients with up to 1, 2, and 3 unfavorable markers (P< .0001). It allowed improved prognostic stratification of ID-PCNSL as compared with the Memorial Sloan Kettering Cancer Center and International Extranodal Lymphoma Study Group models developed for immunocompetent PCNSL. Collectively, this large international cohort defines clinicobiological features of ID-PCNSL and introduces a prognostic system with potential to guide future management.

Indexed as

Central Nervous System NeoplasmsLymphoma, Large B-Cell, DiffuseAdultEpstein-Barr Virus InfectionsFemaleHerpesvirus 4, HumanHumansLymphomaMaleMiddle AgedPrognosisRetrospective StudiesRituximabRituximab

Identifiers

PMID41843467
PMCPMC13386035

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.