B-cell prolymphocytic leukemia
diseaseOn this page
Also known as B prolymphocytic leukaemiaB prolymphocytic leukemiaB-PLLBPLL
Summary
B-cell prolymphocytic leukemia (MONDO:0019461) is a cancer and 11 clinical trials. Top therapeutic interventions include fludarabine phosphate, bendamustine hydrochloride, and letermovir. A subtype of prolymphocytic leukemia — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
- Clinical trials: 11
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.05 | Europe | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | B-cell prolymphocytic leukemia |
| Mondo ID | MONDO:0019461 |
| EFO | EFO:1000102 |
| MeSH | D054403 |
| Orphanet | 86852 |
| DOID | DOID:0081041 |
| ICD-11 | 1997215876 |
| NCIT | C4753 |
| SNOMED CT | 277619001 |
| UMLS | C0475801 |
| MedGen | 105396 |
| GARD | 0008223 |
| Is cancer (heuristic) | yes |
Also known as: B prolymphocytic leukaemia · B prolymphocytic leukemia · B-cell prolymphocytic leukemia · B-PLL · BPLL
Data availability: 2 cell lines.
Disease family
This is a subtype of prolymphocytic leukemia. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › hematopoietic and lymphoid cell neoplasm › leukemia › chronic leukemia › prolymphocytic leukemia › B-cell prolymphocytic leukemia
Related subtypes (2): splenic manifestation of prolymphocytic leukemia, T-cell prolymphocytic leukemia
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Cyclosporine, Fludarabine Phosphate, Mycophenolate Mofetil.
Clinical trials & evidence
Clinical trials
Clinical trials: 11.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE1/PHASE2 | 4 |
| PHASE1 | 4 |
| PHASE2 | 3 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00060424 | PHASE2 | COMPLETED | Fludarabine Phosphate and Total-Body Irradiation Before Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Chronic Lymphocytic Leukemia or Small Lymphocytic Leukemia |
| NCT01008462 | PHASE2 | COMPLETED | Autologous Peripheral Blood Stem Cell Transplant Followed by Donor Bone Marrow Transplant in Treating Patients With High-Risk Hodgkin Lymphoma, Non-Hodgkin Lymphoma, Multiple Myeloma, or Chronic Lymphocytic Leukemia |
| NCT02819583 | PHASE1/PHASE2 | UNKNOWN | CAR-T Cell Immunotherapy in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma |
| NCT02851589 | PHASE1/PHASE2 | UNKNOWN | Study Evaluating the Efficacy and Safety of PCAR-019 in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma |
| NCT02892695 | PHASE1/PHASE2 | UNKNOWN | PCAR-119 Bridge Immunotherapy Prior to Stem Cell Transplant in Treating Patients With CD19 Positive Leukemia and Lymphoma |
| NCT03010358 | PHASE1/PHASE2 | COMPLETED | Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma |
| NCT04312841 | PHASE2 | COMPLETED | Letermovir for the Prevention of Cytomegalovirus Reactivation in Patients With Hematological Malignancies Treated With Alemtuzumab |
| NCT02153580 | PHASE1 | ACTIVE_NOT_RECRUITING | Cellular Immunotherapy Following Chemotherapy in Treating Patients With Recurrent Non-Hodgkin Lymphomas, Chronic Lymphocytic Leukemia, or B-Cell Prolymphocytic Leukemia |
| NCT03479268 | PHASE1 | ACTIVE_NOT_RECRUITING | Pevonedistat and Ibrutinib in Treating Participants With Relapsed or Refractory CLL or Non-Hodgkin Lymphoma |
| NCT05418088 | PHASE1 | RECRUITING | Genetically Engineered Cells (Anti-CD19/CD20/CD22 CAR T-cells) for the Treatment of Relapsed or Refractory Lymphoid Malignancies |
| NCT01886859 | PHASE1 | COMPLETED | Lenalidomide and Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| FLUDARABINE PHOSPHATE | 4 | 4 |
| BENDAMUSTINE HYDROCHLORIDE | 4 | 1 |
| LETERMOVIR | 4 | 1 |
| OBINUTUZUMAB | 4 | 1 |
| ENTOSPLETINIB | 3 | 1 |
| PEVONEDISTAT | 3 | 1 |
| CHEMBL4789656 | 0 | 1 |