Splenic marginal zone lymphoma

Summary

Splenic marginal zone lymphoma (MONDO:0019462) is a cancer and 133 clinical trials. Top therapeutic interventions include fludarabine phosphate, bendamustine hydrochloride, and yttrium y 90 ibritumomab tiuxetan. A subtype of marginal zone lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
• Clinical trials: 133

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 1 000 000	0.5	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	splenic marginal zone lymphoma
Mondo ID	MONDO:0019462
EFO	EFO:1000550
Orphanet	86854
DOID	DOID:0050750
ICD-11	1002343712
NCIT	C4663
SNOMED CT	763666008
UMLS	C0349632
MedGen	138108
GARD	0019072
MedDRA	10062113
Is cancer (heuristic)	yes

Also known as: marginal zone lymphoma of spleen · marginal zone lymphoma of the spleen · SLVL · SMZL · splenic lymphoma with circulating villous lymphocytes · splenic marginal zone B-cell lymphoma · splenic marginal zone B-cell lymphoma with villous lymphocytes · splenic marginal zone lymphoma · splenic marginal zone lymphoma with villous lymphocytes

Data availability: 6 cell lines.

Disease family

This is a subtype of marginal zone lymphoma. 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: disease › human disease › disease by body system or component › immune system disorder › leukocyte disorder › B-cell neoplasm › B-cell non-Hodgkin lymphoma › indolent B-cell non-Hodgkin lymphoma › marginal zone lymphoma › splenic marginal zone lymphoma

Related subtypes (3): MALT lymphoma, splenic diffuse red pulp small B-cell lymphoma, nodal marginal zone B-cell lymphoma

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

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 133.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	52
PHASE2	41
Not specified	18
PHASE1/PHASE2	16
PHASE3	4
EARLY_PHASE1	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT05735834	PHASE3	ACTIVE_NOT_RECRUITING	Comparison Between Rituximab Plus Zanubrutinib Versus Rituximab Monotherapy in Untreated SMZL Patients
NCT00566332	PHASE3	COMPLETED	Trial Comparing Chlorambucil to Fludarabine in Patients With Advanced Waldenström Macroglobulinemia
NCT00799461	PHASE3	COMPLETED	Internet-Based Program With or Without Telephone-Based Problem-Solving Training in Helping Long-Term Survivors of Hematopoietic Stem Cell Transplant Cope With Late Complications
NCT01701232	PHASE3	COMPLETED	Safety and Efficacy Study of BCD-020 in Therapy of Indolent Non-Hodgkin’s Lymphoma
NCT03198026	PHASE2	ACTIVE_NOT_RECRUITING	Obinutuzumab and Ibrutinib as Front Line Therapy in Treating Patients With Indolent Non-Hodgkin’s Lymphomas
NCT03697512	PHASE2	ACTIVE_NOT_RECRUITING	MALIBU Trial - Combination of Ibrutinib and Rituximab in Untreated Marginal Zone Lymphomas
NCT06796998	PHASE2	RECRUITING	Epcoritamab in Patients With Newly Diagnosed Marginal Zone Lymphoma (MZL)
NCT07583810	PHASE2	NOT_YET_RECRUITING	Non-covalent BTK Inhibitor Nemtabrutinib in Combination With the CD20 Monoclonal Antibody Rituximab for the Treatment of Marginal Zone Lymphoma
NCT00003210	PHASE2	COMPLETED	Interleukin-12 in Treating Patients With Previously Treated Non-Hodgkin’s Lymphoma or Hodgkin’s Disease
NCT00005950	PHASE2	TERMINATED	506U78 in Treating Patients With Recurrent or Refractory Non-Hodgkin’s Lymphoma or T-cell Lymphoma
NCT00006251	PHASE1/PHASE2	COMPLETED	Fludarabine Phosphate, Low-Dose Total-Body Irradiation, and Donor Stem Cell Transplant Followed by Cyclosporine, Mycophenolate Mofetil, Donor Lymphocyte Infusion in Treating Patients With Hematopoietic Cancer
NCT00012298	PHASE1/PHASE2	TERMINATED	Radiolabeled Monoclonal Antibody Plus Rituximab With and Without Filgrastim and Interleukin-11 in Treating Patients With Relapsed or Refractory Non-Hodgkin’s Lymphoma
NCT00026182	PHASE2	COMPLETED	Rituximab and Interleukin-12 in Treating Patients With B-Cell Non-Hodgkin’s Lymphoma
NCT00040846	PHASE2	COMPLETED	Alemtuzumab, Fludarabine Phosphate, and Low-Dose Total Body Irradiation Before Donor Stem Cell Transplantation in Treating Patients With Hematological Malignancies
NCT00049504	PHASE2	COMPLETED	Haploidentical Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancer
NCT00054639	PHASE2	COMPLETED	Oblimersen Sodium and Rituximab in Treating Patients With Recurrent B-cell Non-Hodgkin Lymphoma
NCT00058305	PHASE2	COMPLETED	Bryostatin 1 Plus Vincristine in Treating Patients With Progressive or Relapsed Non-Hodgkin’s Lymphoma After Bone Marrow or Stem Cell Transplantation
NCT00072514	PHASE2	COMPLETED	Gemcitabine Hydrochloride, Carboplatin, Dexamethasone, and Rituximab in Treating Patients With Previously Treated Lymphoid Malignancies
NCT00073918	PHASE2	COMPLETED	Iodine I 131 Tositumomab, Etoposide and Cyclophosphamide Followed by Autologous Stem Cell Transplant in Treating Patients With Relapsed or Refractory Non-Hodgkin’s Lymphoma
NCT00078858	PHASE1/PHASE2	COMPLETED	Mycophenolate Mofetil and Cyclosporine in Reducing Graft-Versus-Host Disease in Patients With Hematologic Malignancies or Metastatic Kidney Cancer Undergoing Donor Stem Cell Transplant
NCT00082888	PHASE2	COMPLETED	Tipifarnib in Treating Patients With Relapsed or Refractory Lymphoma
NCT00088881	PHASE2	TERMINATED	Rituximab, Combination Chemotherapy, and 90-Yttrium Ibritumomab Tiuxetan for Patients With Stage I or II Non-Hodgkin’s Lymphoma
NCT00089011	PHASE2	COMPLETED	Tacrolimus and Mycophenolate Mofetil in Preventing Graft-Versus-Host Disease in Patients Who Have Undergone Total-Body Irradiation With or Without Fludarabine Phosphate Followed by Donor Peripheral Blood Stem Cell Transplant for Hematologic Cancer
NCT00089076	PHASE1/PHASE2	TERMINATED	MDX-010 in Treating Patients With Recurrent or Refractory Lymphoma
NCT00112723	PHASE1/PHASE2	TERMINATED	Flavopiridol in Treating Patients With Relapsed or Refractory Lymphoma or Multiple Myeloma
NCT00118352	PHASE2	COMPLETED	Alemtuzumab, Fludarabine Phosphate, and Total-Body Irradiation Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer
NCT00119392	PHASE2	COMPLETED	Yttrium Y 90 Ibritumomab Tiuxetan, Fludarabine, Radiation Therapy, and Donor Stem Cell Transplant in Treating Patients With Relapsed or Refractory Non-Hodgkin’s Lymphoma
NCT00244855	PHASE2	COMPLETED	Rituximab and Dexamethasone in Treating Patients With Low-Grade Non-Hodgkin Lymphoma
NCT00288067	PHASE1/PHASE2	TERMINATED	Fenretinide and Rituximab in Treating Patients With B-Cell Non-Hodgkin Lymphoma
NCT00290472	PHASE2	COMPLETED	CCI-779 in Treating Patients With Recurrent or Refractory B-Cell Non-Hodgkin’s Lymphoma or Chronic Lymphocytic Leukemia
NCT00438880	PHASE1/PHASE2	COMPLETED	Agatolimod Sodium, Rituximab, and Yttrium Y 90 Ibritumomab Tiuxetan in Treating Patients With Recurrent or Refractory Non-Hodgkin Lymphoma
NCT00601718	PHASE1/PHASE2	COMPLETED	Vorinostat, Rituximab, Ifosfamide, Carboplatin, and Etoposide in Treating Patients With Relapsed or Refractory Lymphoma or Previously Untreated T-Cell Non-Hodgkin Lymphoma or Mantle Cell Lymphoma
NCT00711828	PHASE2	COMPLETED	Rituximab, Cyclophosphamide, Bortezomib, and Dexamethasone in Treating Patients With Relapsed or Refractory Low-Grade Follicular Lymphoma, Waldenstrom Macroglobulinemia, or Mantle Cell Lymphoma
NCT00795769	PHASE2	COMPLETED	Ondansetron in Preventing Nausea and Vomiting in Patients Undergoing Stem Cell Transplant
NCT00867529	PHASE2	COMPLETED	Rituximab in Treating Patients Undergoing Donor Peripheral Blood Stem Cell Transplant for Relapsed or Refractory B-cell Lymphoma
NCT00918333	PHASE1/PHASE2	COMPLETED	Panobinostat and Everolimus in Treating Patients With Recurrent Multiple Myeloma, Non-Hodgkin Lymphoma, or Hodgkin Lymphoma
NCT01044745	PHASE2	TERMINATED	Rituximab in Preventing Acute Graft-Versus-Host Disease in a Donor Stem Cell Transplant for Hematologic Cancer
NCT01075321	PHASE1/PHASE2	COMPLETED	Everolimus and Lenalidomide in Treating Patients With Relapsed or Refractory Non-Hodgkin or Hodgkin Lymphoma
NCT01093586	PHASE2	COMPLETED	Donor Umbilical Cord Blood Stem Cell Transplant in Treating Patients With Hematologic Malignancies
NCT01110135	PHASE2	COMPLETED	Bendamustine Hydrochloride, Etoposide, Dexamethasone, and Filgrastim For Peripheral Blood Stem Cell Mobilization in Treating Patients With Refractory or Recurrent Lymphoma or Multiple Myeloma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
FLUDARABINE PHOSPHATE	4	23
BENDAMUSTINE HYDROCHLORIDE	4	5
YTTRIUM Y 90 IBRITUMOMAB TIUXETAN	4	3
ALEMTUZUMAB	4	2
BELINOSTAT	4	2
DEFERASIROX	4	2
PANOBINOSTAT	4	2
BRENTUXIMAB VEDOTIN	4	1
CEFEPIME HYDROCHLORIDE	4	1
CHLORAMBUCIL	4	1
DUVELISIB	4	1
EPCORITAMAB	4	1
FOSCARNET	4	1
FOSCARNET SODIUM	4	1
GANCICLOVIR	4	1
IBRUTINIB	4	1
IDELALISIB	4	1
IPILIMUMAB	4	1
ISOTRETINOIN	4	1
LITHIUM CARBONATE	4	1
NELARABINE	4	1
OBINUTUZUMAB	4	1
OPRELVEKIN	4	1
PALIFERMIN	4	1
ROMIDEPSIN	4	1
TEMSIROLIMUS	4	1
UBLITUXIMAB	4	1
UMBRALISIB	4	1
VALGANCICLOVIR	4	1
VORINOSTAT	4	1