ORPHA:300849
Diffuse large B-cell lymphoma of the central nervous system
Also known as: DLBCL of the CNS
Publications
1,143
Trials
6
Interventional, condition-specific
Researchers
1,422
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0017596
- UMLS:C2026186
- NCIT:C71720
Additional Mondo synonyms (5)
CNS DLBCL · central nervous system diffuse large B-cell lymphoma · diffuse large B-cell lymphoma of central nervous system · primary DLBCL of the CNS · primary diffuse large B-cell lymphoma of the central nervous system
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
1,143 matched papers (925 in last 10 years) Source
- Phenotype characterisedNot found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
6 matched on ClinicalTrials.gov (2 recruiting in sample)
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
12
Drugs / clinical candidates · MONDO_0017596
- CYTARABINE·phase 3
- METHOTREXATE·phase 3
- PROCARBAZINE·phase 3
- RITUXIMAB·phase 3
- EPCORITAMAB·phase 2
- POLATUZUMAB VEDOTIN·phase 2
- POMALIDOMIDE·phase 2
- SELINEXOR·phase 2
- THIOTEPA·phase 2
- ZANUBRUTINIB·phase 2
- ORELABRUTINIB·unknown
- SINTILIMAB·unknown
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
1,143
1,143 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
1,143 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
925 in the last 10 years · low confidence
Phrase hits: 1,143 · MeSH hits: 0
Who's working on it?
1,422
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Li Y9 papers · 2026
Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou, P.R. China.
Papers in Europe PMC - 02Zhang Y9 papers · 2026
First Central Hospital of Tianjin Medical University, Tianjin Medical University, Tianjin, 300070, China.
Papers in Europe PMC - 03Wang Y8 papers · 2025
Tianjin Cancer Hospital, Tianjin Medical University, Tianjin, P.R. China.
Papers in Europe PMC - 04Li J7 papers · 2026
Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China.
Papers in Europe PMC - 05Chen C6 papers · 2026
Department of Biostatistics, GoBroad Research Center, Leeza SOHO South Tower, Fengtai District, Beijing, 100073, China.
Papers in Europe PMC - 06Liu X5 papers · 2025
Department of Radiology, Laigang Hospital Affiliated to Taishan Medical University, Laiwu, Shandong, China (mainland).
Papers in Europe PMC - 07Miao Y5 papers · 2026
Department of Hematology, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
Papers in Europe PMC - 08
- 09Zhang X5 papers · 2026
School of Medicine, Nankai University, Tianjin, 300071, China.
Papers in Europe PMC - 10Guo Y4 papers · 2026
School of Biomedical Engineering, Tsinghua University, Beijing 100084, China. Electronic address: yongguo@tsinghua.edu.cn.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
6
interventional trials for this specific condition
6 interventional trials matched this specific condition name; 2 currently recruiting in our sample. 1,017 trials are registered for diffuse large B-cell lymphoma, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 11 September 2026
6 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 90.1th percentile).
low confidence · 90.1th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
6 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT04609046·RECRUITING·Testing the Addition of Lenalidomide and Nivolumab to the Usual Treatment for Primary CNS Lymphoma
Not reviewed·Conditions: Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System·Matched via name phrase
- NCT07523737·RECRUITING·Pomalidomide, Anti-PD-1 Antibody Combined With Selinexor (PPS) in Relapsed/Refractory Primary Central Nervous System Diffuse Large B-Cell Lymphoma
Not reviewed·Conditions: Primary Central Nervous System Lymphoma (PCNSL) · Diffuse Large B Cell Lymphoma (DLBCL)·Matched via name phrase
Broader category: diffuse large B-cell lymphoma
1,017
Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.
Worth raising with a clinician. How we count trials.
Recruiting under the broader category
- NCT05281809·RECRUITING·Local Manufacture of CAR T-Cell Products for the Treatment of B-Cell Lymphoma and B-Acute Lymphoblastic Leukemia
Not reviewed·Conditions: B-Cell Lymphoma · B Acute Lymphoblastic Leukemia · Diffuse Large B Cell Lymphoma · Mantle Cell Lymphoma·Matched via name phrase
- NCT04491370·RECRUITING·Autologous Stem Cell Transplant Followed by Polatuzumab Vedotin in Patients With B-cell Non-Hodgkin and Hodgkin Lymphoma
Not reviewed·Conditions: B-cell Lymphoma · Burkitt Lymphoma · Diffuse Large B Cell Lymphoma · Follicular Lymphoma·Matched via name phrase
- NCT07507318·RECRUITING·Chidamide Maintenance for MRD-Positive Double-Expressor DLBCL in First Complete Remission
Not reviewed·Conditions: Diffuse Large B-Cell Lymphoma·Matched via name phrase
- NCT07570823·NOT YET RECRUITING·A Study to Evaluate the Safety and Efficacy of SCTB35 in Combination With Gemcitabine and Oxaliplatin in Patients With Relapsed or Refractory Diffuse Large B-Cell Lymphoma
Not reviewed·Conditions: DLBCL - Diffuse Large B Cell Lymphoma·Matched via name phrase
- NCT06352242·RECRUITING·Is Trogocytosis a Predictive Marker of CAR-T Cell Response in Diffuse Large B-cell Lymphoma?
Not reviewed·Conditions: Diffuse Large B Cell Lymphoma·Matched via name phrase
- NCT07001540·RECRUITING·Evaluation of the Efficacy and Safety of Polatuzumab Vedotin Combined With Rituximab, Gemcitabine, and Oxaliplatin (Pola-R-GemOx) as Salvage Therapy for Relapsed/Refractory Diffuse Large B-Cell Lymphoma (DLBCL) Patients Ineligible for Autologous Transplantation
Not reviewed·Conditions: Diffuse Large B-Cell Lymphoma·Matched via name phrase
- NCT03676504·RECRUITING·Treatment of Patients With Relapsed or Refractory CD19+ Lymphoid Disease With T Cells Expressing a Third-generation CAR
Not reviewed·Conditions: Acute Lymphoblastic Leukemia, Adult · Acute Lymphoblastic Leukemia, Pediatric · Chronic Lymphocytic Leukemia · Diffuse Large B Cell Lymphoma·Matched via name phrase
- NCT06569485·RECRUITING·A Study of Trilaciclib Combined With Chemotherapy in the Treatment of Diffuse Large B-Cell Lymphoma Patients
Not reviewed·Conditions: DLBCL·Matched via name phrase
- NCT06570447·RECRUITING·Glofitamab Combination With Chidamide in Patients With Recurrent/Refractory DLBCL
Not reviewed·Conditions: Diffuse Large B-Cell Lymphoma-Recurrent · Diffuse Large B-Cell Lymphoma-Refractory·Matched via name phrase
- NCT05641428·RECRUITING·Comparison of Point-of-care Produced CAR T-cell with Commercial CAR T-cells in Patients with R/R LBCL
Not reviewed·Conditions: NHL · DLBCL - Diffuse Large B Cell Lymphoma·Matched via name phrase
- NCT06830031·RECRUITING·Clinical Study of C402-CD19-CAR Treatment in Subjects With Relapsed or Refractory B-cell Lymphoma
Not reviewed·Conditions: Diffuse Large B-cell-lymphoma · DLBCL, Nos Genetic Subtypes · Follicular Lymphoma Grade 3B · PMBL·Matched via name phrase
- NCT06977711·RECRUITING·Loncastuximab and Roflumilast Added to R-CHOP (Lo-(Rituximab and Roflumilast) RR-CHOP) for Naïve High-Risk Diffuse Large B-cell Lymphoma (DLBCL)
Not reviewed·Conditions: Diffuse Large B-cell Lymphoma·Matched via name phrase
- NCT06652139·NOT YET RECRUITING·Selinexor Combined With BEAM for ASCT in R/R DLBCL With MYC Positive
Not reviewed·Conditions: Diffuse Large B Cell Lymphoma·Matched via name phrase
- NCT07124936·RECRUITING·A Study of HDM2005 in Combination With Standard of Care in Patients With Diffuse Large B-Cell Lymphoma
Not reviewed·Conditions: Diffuse Large B Cell Lymphoma (DLBCL)·Matched via name phrase
- NCT07164469·NOT YET RECRUITING·Phase 2 Trial of CD70.CAR NK Cells for Patients With Primary Refractory or Early Relapsed Diffuse Large B-Cell Lymphoma and Hodgkin Lymphoma
Not reviewed·Conditions: Large B-cell Lymphoma · Hodgkin Lymphoma·Matched via name phrase
Observational and natural-history studies
3 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
- NCT07147751·NOT YET RECRUITING·Characterization of Primary Central Nervous System Diffuse Large B Cell Lymphoma (PCNS-DLBCL) by Multiomic Approach
Not reviewed·Conditions: Primary Central Nervous System Diffuse Large B Cell Lymphoma (PCNS-DLBCL)·Matched via name phrase
- NCT07188077·RECRUITING·Metabolic and Immunomic Differential Analysis of Primary Central Nervous System Diffuse Large B-Cell Lymphoma
Not reviewed·Conditions: Primary Central Nervous System Lymphoma (PCNSL) · Diffuse Large B-Cell Lymphoma·Matched via name phrase
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 28 · after dedupe 28 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 28 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (28)
- ctis·2024-512320-11-00·Authorised, ongoing·Age-adjusted high-dose chemotherapy followed by autologous stem cell transplantation or conventional chemotherapy with R-MP as first-line treatment in elderly primary CNS lymphoma patients – a randomized phase III trial - PRIMA-CNS
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN10023611·Recruiting·A study of JNJ-80948543 in combination with other CD3 T-cell engagers in participants with relapsed/refractory B-cell non-Hodgkin lymphoma (R/R B-Cell NHL)
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN15038320·Recruiting·A phase 1b/2 study of JNJ-90014496 in Adult Participants with B-Cell non-Hodgkin lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN17666983·No longer recruiting·A Study of JNJ-90009530 in Relapsed or Refractory B-Cell non-Hodgkin Lymphoma (r/r B-NHL)
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN14742241·No longer recruiting·Optimizing the MATRix chemotherapy regimen, to reduce side-effects and improve outcomes, for patients with newly diagnosed primary central nervous system lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN13438605·No longer recruiting·A trial looking at the effectiveness of combining standard R-ICE chemotherapy with another medicine (polatuzumab vedotin) for patients with diffuse large B cell lymphoma that has either, not responded to or returned, following the first treatment received
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN90634455·Recruiting·A trial of zanubrutinib treatment of patients with relapsed and refractory primary central nervous system lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN14251143·No longer recruiting·A trial evaluating the effectiveness of combining standard R-CHOP treatment with acalabrutinib in patients with newly diagnosed diffuse large B-cell lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN11542980·No longer recruiting·Evaluating alternative treatment regimens for patients who have diffuse large B-cell lymphoma that is unsuitable for standard treatment
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN17568633·No longer recruiting·A study to evaluate safety, interaction with the body (pharmacokinetics), and clinical activity of combination of RO6870810 and venetoclax, with or without rituximab, in participants with cancer of the white blood cells (lymphoma)
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN15025004·No longer recruiting·A phase IIa study of rituximab and varlilumab in relapsed or refractory B-cell malignancies
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN13626902·No longer recruiting·The ACCEPT study
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN10760016·No longer recruiting·A study of the drugs AZD2014 and rituximab in relapsed or refractory diffuse large B-cell lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN12857473·No longer recruiting·Investigating the most appropriate dose and effectiveness of thiotepa in combination with ifosphamide, etoposide and rituximab in patients with lymphoma arising in the brain or spinal cord
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN26918647·No longer recruiting·Study of RTXM83 plus CHOP chemotherapy versus a rituximab plus CHOP therapy in patients with Non Hodgkin’s lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN66315383·Stopped·A study of GCS-100 in combination with chemo-immunotherapy in patients with diffuse large B-cell lymphoma which have relapsed or are refractory to treatment
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN82286322·No longer recruiting·Randomised phase III study on the effect of early intensification of rituximab in combination with two-weekly cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) chemotherapy followed by rituximab maintenance in elderly patients (66 to 80 years) with diffuse large B-cell lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN16017947·No longer recruiting·A phase III multicentre randomised clinical trial comparing rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone given every 14 days and rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone given every 21 days for the treatment of patients with newly diagnosed diffuse large B cell non-Hodgkin's lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN95614846·No longer recruiting·A randomised phase III study on the effect of the chimeric anti-CD20 monoclonal antibody (MabThera®) during sequential chemotherapy followed by autologous stem cell transplantation in patients with relapsed or progressive B-cell non-Hodgkin’s lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN84611849·No longer recruiting·A randomised phase III study of chimeric anti-CD20 monoclonal antibody (rituximab) with two-weekly CHOP chemotherapy (CHOP 14) in elderly patients with intermediate or high-risk non-Hodgkin’s lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN15438979·Recruiting·An early phase trial to test the safety and determine the appropriate dose of BTM-3566 in patients with mature B cell lymphoma and advanced solid tumors
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN13777452·Recruiting·Study of DTP3 in patients with relapsed or refractory multiple myeloma or diffuse large B-cell lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN86607306·No longer recruiting·Pembrolizumab plus chemotherapy for diffuse large B-cell lymphoma that has come back or does not respond to treatment
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN52839057·No longer recruiting·A trial of CHOP-R therapy, with or without acalabrutinib, in patients with newly diagnosed Richter's Syndrome
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN11965217·No longer recruiting·A trial to assess whether the addition of atezolizumab to current standard treatment for patients with relapsed or refractory Diffuse Large B-Cell Lymphoma, who are not able to have high dose therapy, improves survival outcomes
skipped — LLM skipped (--skip-llm)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Diffuse large B-cell lymphoma of the central nervous system — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Diffuse large B-cell lymphoma of the central nervous system" OR "Diffuse large B-cell lymphoma of central nervous system" OR "DLBCL of the CNS" OR "DLBCL of CNS" OR "CNS DLBCL" OR "central nervous system diffuse large B-cell lymphoma" OR "primary DLBCL of the CNS" OR "primary DLBCL of CNS" OR "primary diffuse large B-cell lymphoma of the central nervous system" OR "primary diffuse large B-cell lymphoma of central nervous system"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Diffuse large B-cell lymphoma of the central nervous system" OR "Diffuse large B-cell lymphoma of central nervous system" OR "DLBCL of the CNS" OR "DLBCL of CNS" OR "CNS DLBCL" OR "central nervous system diffuse large B-cell lymphoma" OR "primary DLBCL of the CNS" OR "primary DLBCL of CNS" OR "primary diffuse large B-cell lymphoma of the central nervous system" OR "primary diffuse large B-cell lymphoma of central nervous system"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 6 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"diffuse large B-cell lymphoma"
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
- Publication count (1143) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T12:43:33.366Z
