Splenic Marginal Zone Lymphoma disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

SMZL MCID: SPL004 MIFTS: 48	Splenic Marginal Zone Lymphoma (SMZL) Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
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Aliases & Classifications for Splenic Marginal Zone Lymphoma

MalaCards integrated aliases for Splenic Marginal Zone Lymphoma:

Name: Splenic Marginal Zone Lymphoma ¹¹ ⁵⁸ ¹⁴ ³⁶ ¹⁶ ⁷⁵

Splenic Marginal Zone B-Cell Lymphoma ⁷¹

Smzl ⁵⁸

Characteristics:

Prevelance:

1-9/1000000 (Europe) ⁵⁸

Age Of Onset:

Adult ⁵⁸

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Blood diseases Immune diseases Bone diseases

See all MalaCards categories (disease lists)

ICD10: ³²

Neoplasms

Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue

Non-follicular lymphoma

Small cell B-cell lymphoma

Orphanet: ⁵⁸

Rare haematological diseases

External Ids:

Disease Ontology ¹¹ DOID:0050750

ICD10 via Orphanet ³² C83.0

UMLS via Orphanet ⁷² C0349632

Orphanet ⁵⁸ ORPHA86854

EFO ¹⁶ EFO_1000550

UMLS ⁷¹ C0349632

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Summaries for Splenic Marginal Zone Lymphoma

Orphanet: ⁵⁸ Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated.

MalaCards based summary: Splenic Marginal Zone Lymphoma, also known as splenic marginal zone b-cell lymphoma, is related to burkitt lymphoma and lymphoproliferative syndrome. An important gene associated with Splenic Marginal Zone Lymphoma is PAX5 (Paired Box 5), and among its related pathways/superpathways are Innate Immune System and NF-kappaB Signaling. The drugs Chlorambucil and Ondansetron have been mentioned in the context of this disorder. Affiliated tissues include spleen comprised of b-cells in place of white pulp, bone marrow and b cells, and related phenotypes are Synthetic lethal with vaccinia virus (VACV) infection and Reduced mammosphere formation

Disease Ontology: ¹¹ A marginal zone B-cell lymphocyte located in the spleen comprised of B-cells in place of white pulp.

Wikipedia: ⁷⁵ Splenic marginal zone lymphoma (SMZL) is a type of cancer (specifically a lymphoma) made up of B-cells... more...

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Related Diseases for Splenic Marginal Zone Lymphoma

Diseases related to Splenic Marginal Zone Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 277)

#	Related Disease	Score	Top Affiliating Genes
1	burkitt lymphoma	31.7	PAX5 MME CD19 BCL6
2	lymphoproliferative syndrome	31.4	CD5 CCR6 BCL6
3	lymphoma	31.4	PAX5 MYD88 CD5 CD19 CCR6 CARD11
4	follicular lymphoma	31.1	PAX5 MME CD5 BCL6
5	anemia, autoimmune hemolytic	31.1	IGHV4-38-2 CD5 CD19 CCR6
6	lymphoma, hodgkin, classic	31.0	TRAF3 PAX5 IL3RA CCR6 BCL6
7	b-cell lymphoma	31.0	PAX5 MYD88 MME CD79B CD5 CD22
8	deficiency anemia	30.8	U2AF1 MME CD5 CD19 CCR6
9	thrombocytopenia due to platelet alloimmunization	30.8	U2AF1 CD19 CCR6
10	hematologic cancer	30.7	U2AF1 PAX5 IL3RA CCR6
11	waldenstroem's macroglobulinemia	30.7	PAX5 MYD88 IGHV4-38-2 CD79B CD5 CD19
12	pancytopenia	30.7	U2AF1 MME IL3RA CD5 CD22 CD19
13	gamma heavy chain disease	30.6	PAX5 IGHV4-38-2 CD19
14	orofaciodigital syndrome viii	30.6	IGHV4-38-2 CD5 BCL6
15	nodal marginal zone lymphoma	30.5	SPN MME IGHV4-38-2 CD79A CD5 BCL6
16	reticulosarcoma	30.5	PAX5 CD79A BCL6
17	heavy chain disease	30.5	MYD88 IGHV4-38-2 CD79A
18	agammaglobulinemia	30.5	CD79B CD79A CD19
19	isolated growth hormone deficiency	30.5	U2AF1 IGHV4-38-2 CCR6
20	evans' syndrome	30.5	U2AF1 CD19 CCR6
21	splenic infarction	30.4	U2AF1 CD5
22	marginal zone b-cell lymphoma	30.3	U2AF1 SPN PAX5 NOTCH2 MYD88 MME
23	hairy cell leukemia	30.3	U2AF1 MME ITGAX ITGAE IL3RA IGHV4-38-2
24	agammaglobulinemia, x-linked	30.2	CD79B CD79A CD19 CCR6
25	composite lymphoma	30.1	SPN PAX5 MME IGHV4-38-2 CD79A CD5
26	lymphatic system disease	29.9	U2AF1 MME IGHV4-38-2 CD5 CD19 CCR6
27	immune deficiency disease	29.9	SPN CD79A CD5 CCR6 BCL6
28	myelodysplastic syndrome	29.8	U2AF1 SPN IL3RA CD19 CCR6
29	diffuse large b-cell lymphoma	29.8	U2AF1 PAX5 MYD88 MME IGHV4-38-2 CD79B
30	mantle cell lymphoma	29.5	U2AF1 SPN PAX5 MYD88 MME IGHV4-38-2
31	chronic lymphocytic leukemia/small lymphocytic lymphoma	29.5	SPN PAX5 MME IGHV4-38-2 CD79B CD79A
32	lymphoplasmacytic lymphoma	29.4	SPN PAX5 MYD88 MME IGHV4-38-2 CD79B
33	lymphoma, non-hodgkin, familial	29.3	U2AF1 SPN PAX5 MYD88 MME ITGAX
34	mature b-cell neoplasm	29.3	U2AF1 SPN PAX5 MME IGHV4-38-2 CD79B
35	myeloma, multiple	29.3	U2AF1 TRAF3 PAX5 MYD88 CD79B CD79A
36	splenic disease	29.3	U2AF1 MYD88 MME ITGAX ITGAE IGHV4-38-2
37	lymphoma, mucosa-associated lymphoid type	29.1	TRAF3 SPN PAX5 MYD88 MME IGHV4-38-2
38	leukemia, chronic lymphocytic	28.8	U2AF1 SPN PAX5 NOTCH2 MYD88 MME
39	leukemia, acute myeloid	28.5	U2AF1 SPN PAX5 MME ITGAX IL3RA
40	splenomegaly	11.0
41	hemolytic anemia	10.7
42	monosomy 7 myelodysplasia and leukemia syndrome 1	10.6
43	hepatitis b	10.6
44	hepatitis	10.6
45	viral infectious disease	10.6
46	aplastic anemia	10.5
47	hepatitis c virus	10.5
48	thrombocytopenia	10.5
49	hepatitis c	10.5
50	macroglobulinemia	10.5

Graphical network of the top 20 diseases related to Splenic Marginal Zone Lymphoma:

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Symptoms & Phenotypes for Splenic Marginal Zone Lymphoma

GenomeRNAi Phenotypes related to Splenic Marginal Zone Lymphoma according to GeneCards Suite gene sharing:

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Synthetic lethal with vaccinia virus (VACV) infection	GR00362-A	9.35	BCL6 CD79A CD79B MME TRAF3
2	Reduced mammosphere formation	GR00396-S	9.17	BCL6 CCR6 CD22 CD5 CD79A IL3RA

MGI Mouse Phenotypes related to Splenic Marginal Zone Lymphoma:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	normal	MP:0002873	10.02	BCL6 CCR6 CD19 CD5 CD79A CD79B
2	immune system	MP:0005387	9.93	BCL6 CARD11 CCR6 CD19 CD22 CD5
3	neoplasm	MP:0002006	9.91	CD19 CD79A MYD88 NOTCH2 PAX5 SPN
4	no phenotypic analysis	MP:0003012	9.91	BCL6 CD5 CD79A CD79B ITGAX KLF2
5	liver/biliary system	MP:0005370	9.76	BCL6 CD19 CD79A KLF2 MME MYD88
6	hematopoietic system	MP:0005397	9.58	BCL6 CARD11 CCR6 CD19 CD22 CD5

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Drugs & Therapeutics for Splenic Marginal Zone Lymphoma

Drugs for Splenic Marginal Zone Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 154)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Chlorambucil

Approved

Phase 3

305-03-3

2708

Ondansetron

Approved, Withdrawn

Phase 2

99614-02-5

4595

Carboplatin

Approved

Phase 1, Phase 2

41575-94-4

10339178 38904

Melphalan

Approved

Phase 2

148-82-3

4053 460612

Mechlorethamine

Approved, Investigational

Phase 2

51-75-2

4033

Vorinostat

Approved, Investigational

Phase 1, Phase 2

149647-78-9

5311

Dexamethasone acetate

Approved, Investigational, Vet_approved

Phase 2

1177-87-3

3680

Dexamethasone

Approved, Investigational, Vet_approved

Phase 2

50-02-2

3003 5743

Iodine

Approved, Investigational

Phase 2

7553-56-2

807

Miconazole

Approved, Investigational, Vet_approved

Phase 1, Phase 2

22916-47-8

4189

Clotrimazole

Approved, Vet_approved

Phase 1, Phase 2

23593-75-1

2812

Prednisolone phosphate

Approved, Vet_approved

Phase 2

302-25-0

Prednisolone acetate

Approved, Vet_approved

Phase 2

52-21-1

Prednisolone

Approved, Vet_approved

Phase 2

50-24-8

4894 5755

Methylprednisolone hemisuccinate

Approved

Phase 2

2921-57-5

1875

Methylprednisolone

Approved, Vet_approved

Phase 2

83-43-2

4159 6741

Ifosfamide

Approved

Phase 1, Phase 2

3778-73-2

3690

Panobinostat

Approved, Investigational

Phase 1, Phase 2

404950-80-7

6918837

Everolimus

Approved

Phase 1, Phase 2

159351-69-6

70789204 6442177

Sirolimus

Approved, Investigational

Phase 1, Phase 2

53123-88-9

5284616 6436030

Alemtuzumab

Approved, Investigational

Phase 2

216503-57-0

Levoleucovorin

Approved, Experimental, Investigational

Phase 2

68538-85-2, 58-05-9, 73951-54-9

149436 6006

Methotrexate

Approved

Phase 2

1959-05-2, 59-05-2

4112 126941

Obinutuzumab

Approved, Investigational

Phase 2

949142-50-1

Thiotepa

Approved, Investigational

Phase 2

52-24-4

5453

Idelalisib

Approved

Phase 2

870281-82-6

11654566 11625818

Pembrolizumab

Approved

Phase 2

1374853-91-4

254741536

Etoposide

Approved

Phase 1, Phase 2

33419-42-0

36462

Lenograstim

Approved, Investigational

Phase 1, Phase 2

135968-09-1

Busulfan

Approved, Investigational

Phase 2

55-98-1

2478

Ipilimumab

Approved

Phase 1, Phase 2

477202-00-9

Vincristine

Approved, Investigational

Phase 2

2068-78-2, 57-22-7

5978

Prednisone

Approved, Vet_approved

Phase 2

53-03-2

5865

Doxorubicin

Approved, Investigational

Phase 2

23214-92-8

31703

Oprelvekin

Approved, Investigational

Phase 1, Phase 2

145941-26-0

Iron

Approved

Phase 2

7439-89-6

29936

Deferasirox

Approved, Investigational

Phase 2

201530-41-8

214348 5493381

Brentuximab vedotin

Approved, Investigational

Phase 1, Phase 2

914088-09-8

D-Phenylalanine

Approved, Experimental, Investigational, Nutraceutical

Phase 2

63-91-2, 673-06-3

6140 71567

Folic acid

Approved, Nutraceutical, Vet_approved

Phase 2

59-30-3

6037

Cadexomer iodine

Experimental

Phase 2

94820-09-4

Prednisolone hemisuccinate

Experimental

Phase 2

2920-86-7

4897

Palifosfamide

Investigational

Phase 1, Phase 2

31645-39-3

100427

Tipifarnib

Investigational

Phase 2

192185-72-1

159324

Bryostatin 1

Investigational

Phase 2

83314-01-6

Veliparib

Investigational

Phase 1, Phase 2

912444-00-9

11960529

Alvocidib

Experimental, Investigational

Phase 1, Phase 2

131740-09-5, 146426-40-6

5466794 5287969

Fenretinide

Investigational

Phase 1, Phase 2

65646-68-6

5288209

Antipsychotic Agents

Phase 2

Anti-Anxiety Agents

Phase 2

Interventional clinical trials:

(show top 50) (show all 132)

#	Name	Status	NCT ID	Phase	Drugs
1	Randomized Trial Comparing the Efficacy of Chlorambucil to Fludarabine in Patients With Advanced Waldenström Macroglobulinemia,Lymphoplasmacytic Lymphoma or Splenic Marginal Zone Lymphoma	Completed	NCT00566332	Phase 3	Chlorambucil;Fludarabine
2	INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation	Completed	NCT00799461	Phase 3
3	A Multicenter Open-label Randomized Study of BCD-020 (Rituximab, CJSC BIOCAD, Russia) Efficacy and Safety in Comparison With MabThera (F. Hoffmann-La Roche Ltd., Switzerland) in Monotherapy of CD20-positive Indolent Non-Hodgkin's Lymphoma	Completed	NCT01701232	Phase 3
4	Bendamustine and Rituximab for the Treatment of Splenic Marginal Zone Lymphoma. The International Extranodal Lymphoma Study Group (IELSG) 36 Phase II Prospective Study	Completed	NCT02853370	Phase 2	Bendamustine and Rituximab
5	Pilot Study of Prophylactic Dose-Escalation Donor Lymphocyte Infusion After T Cell Depleted Allogeneic Stem Cell Transplant in High Risk Patients With Hematologic Malignancies	Completed	NCT01839916	Phase 2
6	Addition of Pre- and Post-Transplant Rituximab for Patients Undergoing Non-myeloablative Allogeneic Hematopoietic Cell Transplantation With Relapsed or Refractory CD20+ B-Cell Malignancies	Completed	NCT00867529	Phase 2
7	Prevention of DMSO-Related Nausea and Vomiting by Prophylactic Administration of Ondansetron for Patients Receiving Autologous Cryopreserved Peripheral Blood Stem Cells	Completed	NCT00795769	Phase 2	ondansetron
8	A Phase I/II Study of Intratumoral Injection of SD-101, an Immunostimulatory CpG, and Intratumoral Injection of Ipilimumab, an Anti-CTLA4 Monoclonal Antibody, in Combination With Local Radiation in Low-Grade B-Cell Lymphomas	Completed	NCT02254772	Phase 1, Phase 2	SD-101
9	Phase II Clinical Trial of Rituximab in Combination With Pegfilgrastim in Patients With Indolent B-Cell (CD-20-Positive) Lymphoma	Completed	NCT01682044	Phase 2
10	A Phase 2 Clinical Trial of Rituximab, Cyclophosphamide, Bortezomib (VELCADE), and Dexamethasone (R-CYBOR-D) in Relapsed Low Grade and Mantle Cell Lymphoma	Completed	NCT00711828	Phase 2	Bortezomib;Cyclophosphamide;Dexamethasone
11	A Phase I/II Trial of CpG 7909, Rituximab Immunotherapy, and Y-90 Zevalin Radioimmunotherapy for Patients With Previously Treated CD20+ Non-Hodgkin Lymphoma	Completed	NCT00438880	Phase 1, Phase 2	Agatolimod Sodium
12	A Phase II Trial Evaluating The Efficacy of Radioiodinated Tositumomab (Anti-CD20) Antibody, Etoposide and Cyclophosphamide Followed by Autologous Transplantation, for Relapsed or Refractory Non-Hodgkin's Lymphoma	Completed	NCT00073918	Phase 2	cyclophosphamide;etoposide
13	Prolonged Mycophenolate Mofetil and Truncated Cyclosporine Postgrafting Immunosuppression to Reduce Life-Threatening GVHD After Unrelated Donor Peripheral Blood Cell Transplantation Using Nonmyeloablative Conditioning for Patients With Hematologic Malignancies and Renal Cell Carcinoma - A Multi-Center Trial	Completed	NCT00078858	Phase 1, Phase 2	fludarabine phosphate;cyclosporine;mycophenolate mofetil
14	A Phase II Study of G3139 (Bcl-2 Antisense) And Rituximab in Patients With Recurrent B-cell Non-Hodgkinâs Lymphomas	Completed	NCT00054639	Phase 2
15	Randomized Phase II Study Of Interleukin-12 In Combination With Rituximab In Patients With Non-Hodgkin's Lymphoma	Completed	NCT00026182	Phase 2
16	A Phase II Study of Bendamustine (B), Etoposide (E), Dexamethasone (D), and GCSF for Peripheral Blood Hematopoietic Stem Cell Mobilization (BED)	Completed	NCT01110135	Phase 2	bendamustine hydrochloride;dexamethasone;etoposide
17	A Phase II Trial Evaluating the Safety and Efficacy of Non-myeloablative 90Y-Ibritumomab Tiuxetan (Anti-CD20) Antibody With Fludarabine, Low-Dose Total Body Irradiation (TBI) and HLA Matched Allogeneic Transplantation for Relapsed B-cell Lymphoma	Completed	NCT00119392	Phase 2	cyclosporine;fludarabine phosphate;mycophenolate mofetil
18	A Multi-Center Study of Nonmyeloablative Conditioning With TBI or Fludarabine/TBI for HLA-matched Related Hematopoietic Cell Transplantation for Treatment of Hematologic Malignancies With Post Grafting Immunosuppression With Tacrolimus and Mycophenolate Mofetil	Completed	NCT00089011	Phase 2	fludarabine phosphate;mycophenolate mofetil;tacrolimus
19	Induction of Mixed Hematopoietic Chimerism in Patients Using Fludarabine, Low Dose TBI, PBSC Infusion and Post-Transplant Immunosuppression With Cyclosporine and Mycophenolate Mofetil	Completed	NCT00006251	Phase 1, Phase 2	fludarabine phosphate;cyclosporine;mycophenolate mofetil
20	Umbilical Cord Blood (UCB) Allogeneic Stem Cell Transplant for Hematologic Malignancies	Completed	NCT01093586	Phase 2	busulfan;cyclophosphamide;anti-thymocyte globulin;methylprednisolone;cyclosporine;mycophenolate mofetil
21	Nonmyeloablative Hematopoietic Stem Cell Transplantation for Patients With High-Risk Hematologic Malignancies Using Related, HLA-Haploidentical Donors: A Phase II Trial of Combined Immunosuppression Before and After Transplantation	Completed	NCT00049504	Phase 2	cyclophosphamide;fludarabine phosphate;tacrolimus;mycophenolate mofetil
22	A Phase I/II Study of Vorinostat Plus Rituximab, Ifosphamide, Carboplatin, and Etoposide for Patients With Relapsed or Refractory Lymphoid Malignancies or Untreated T- or Mantle Cell Lymphoma	Completed	NCT00601718	Phase 1, Phase 2	vorinostat;ifosfamide;carboplatin;etoposide
23	A Phase I/II Study of the Histone Deacetylase (HDAC) Inhibitor LBH589 (Panobinostat) in Combination With mTOR Inhibitor RAD001 (Everolimus) in Patients With Relapsed Multiple Myeloma or Lymphoma	Completed	NCT00918333	Phase 1, Phase 2	panobinostat;everolimus
24	A Phase II Study of CCI-779 in B-cell Lymphoma and CLL	Completed	NCT00290472	Phase 2	temsirolimus
25	Rituximab and Dexamethasone in CD20 Positive Low Grade and Follicular Non-Hodgkin's Lymphoma	Completed	NCT00244855	Phase 2	dexamethasone
26	Campath® [Alemtuzumab] Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class I Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies - A Multi-Center Trial	Completed	NCT00040846	Phase 2	fludarabine phosphate;mycophenolate mofetil;cyclosporine
27	A Phase I/II Clinical Trial of the mTor Inhibitor RAD001 (Everolimus) in Combination With Lenalidomide (Revlimid) for Patients With Relapsed or Refractory Lymphoid Malignancy	Completed	NCT01075321	Phase 1, Phase 2	everolimus;lenalidomide
28	Campath (Alemtuzumab) Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class II Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies: A Multicenter Trial	Completed	NCT00118352	Phase 2	fludarabine phosphate;cyclosporine;mycophenolate mofetil
29	Phase II Evaluation of FTI (R115777) in Treatment of Relapsed and Refractory Lymphoma	Completed	NCT00082888	Phase 2	Tipifarnib
30	A Phase II Study Evaluating the Efficacy of Gemcitabine, Carboplatin, and Dexamethasone and Rituximab for Previously Treated Lymphoid Malignancies	Completed	NCT00072514	Phase 2	gemcitabine hydrochloride;carboplatin;dexamethasone
31	A Phase II Study of Bryostatin 1 and Vincristine in Patients With Low or Intermediate Grade Non-Hodgkin's Lymphoma Progressing or Relapsing After a Prior Autologous Bone Marrow or Stem Cell Transplant	Completed	NCT00058305	Phase 2	bryostatin 1;vincristine sulfate
32	A Phase II Study of Recombinant Human Interleukin-12 (rhIL-12) for the Treatment of Relapsed Lymphoma and Hodgkin's Disease	Completed	NCT00003210	Phase 2
33	Phase II Study of MK-2206 in Patients With Relapsed Lymphoma	Completed	NCT01258998	Phase 2	Akt inhibitor MK2206
34	A Phase II Study of the Histone Deacetylase (HDAC) Inhibitor LBH589 (Panobinostat) in Patients With Relapsed or Refractory Non-Hodgkin Lymphoma	Completed	NCT01261247	Phase 2	panobinostat
35	A Phase II Trial of Reduced Intensity Allogeneic Stem Cell Transplantation With Fludarabine, Melphalan and Low Dose Total Body Irradiation	Completed	NCT01529827	Phase 2	fludarabine phosphate;melphalan;tacrolimus;mycophenolate mofetil;methotrexate
36	A Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for Patients With Hematologic Malignancies	Completed	NCT01384513	Phase 2	Fludarabine;Busulfan;Cyclophosphamide (CY);Tacrolimus;Mycophenolate mofetil
37	High-dose Busulfan, High-dose Cyclophosphamide, and Allogeneic Bone Marrow Transplantation for Leukemia, Myelodysplastic Syndromes, Multiple Myeloma and Lymphoma	Completed	NCT01177371	Phase 2	busulfan;cyclophosphamide;methylprednisolone;methotrexate;cyclosporine
38	A Pilot Trial of Vorinostat Plus Tacrolimus and Methotrexate to Prevent Graft Versus Host Disease Following Unrelated Donor Hematopoietic Stem Cell Transplantation	Completed	NCT01789255	Phase 2	vorinostat;tacrolimus;cyclosporine;methotrexate
39	A Phase II Study to Evaluate the Efficacy of Posttransplant Cyclophosphamide for Prevention of Chronic Graft-versus-Host Disease After Allogeneic Peripheral Blood Stem Cell Transplantation	Completed	NCT01427881	Phase 2	cyclophosphamide;cyclosporine;fludarabine phosphate;busulfan
40	A Phase 1b/2a Study of ABT-888 in Combination With Bendamustine +/- Rituximab in Lymphoma, Multiple Myeloma and Solid Tumors	Completed	NCT01326702	Phase 1, Phase 2	Bendamustine Hydrochloride;Veliparib
41	Impact of Intervention With Deferasirox on the Immune Function of Patients With Hematologic Diseases and Transfusion-Related Iron Overload	Completed	NCT01273766	Phase 2	deferasirox
42	Phase II, Single Arm, Open Label Multi-center Study of Obinutuzumab and Ibrutinib in the Front Line Treatment of Indolent Non-Hodgkin's Lymphomas	Recruiting	NCT03198026	Phase 2	Ibrutinib;Obinutuzumab
43	MALIBU Trial - Phase II Study of Combination Ibrutinib and Rituximab in Untreated Marginal Zone Lymphomas	Recruiting	NCT03697512	Phase 2	Ibrutinib;Rituximab
44	A Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for High Risk Hematologic Malignancies	Recruiting	NCT01760655	Phase 2	Fludarabine phosphate;Thiotepa;Cyclophosphamide;Tacrolimus;Mycophenolate mofetil
45	A Phase II Study of Anti-PD-1 Antibody (MK-3475) in Relapsed/Refractory Chronic Lymphocytic Leukemia (CLL) and Other Low Grade B Cell Non-Hodgkin Lymphoma (NHL)	Active, not recruiting	NCT02332980	Phase 2	Ibrutinib;Idelalisib
46	Prospective, Multi-center Phase I/II Trial of Lenalidomide and Dose-Adjusted EPOCH-R in MYC-Associated B-Cell Lymphomas	Active, not recruiting	NCT02213913	Phase 1, Phase 2	lenalidomide;etoposide;prednisone;vincristine sulfate;doxorubicin hydrochloride;cyclophosphamide
47	A Phase II Trial of R-CHOP Followed by Zevalin Radioimmunotherapy for Patients With Previously Untreated Stages I and II CD20+ Diffuse Large Cell Non-Hodgkin's Lymphoma	Terminated	NCT00088881	Phase 2	prednisone;cyclophosphamide;doxorubicin;vincristine
48	Phase I/II Study of Anti-CTLA-4 Monoclonal Antibody (MDX-010) in B-cell Non-Hodgkin's Lymphoma	Terminated	NCT00089076	Phase 1, Phase 2
49	A Phase I/II Study of Flavopiridol Administered as a 30-Minute Bolus Followed by a 4-Hour Infusion in Lymphomas and Multiple Myeloma	Terminated	NCT00112723	Phase 1, Phase 2	alvocidib
50	Phase II Study of 506U78 (NSC #686673) for Patients With Relapsed or Refractory Indolent B-Cell or Peripheral T-Cell Lymphoma	Terminated	NCT00005950	Phase 2	nelarabine

Search NIH Clinical Center for Splenic Marginal Zone Lymphoma

Cell-based therapeutics:

Data from LifeMap, the Embryonic Development and Stem Cells Database

Read about Splenic Marginal Zone Lymphoma cell therapies at LifeMap Discovery.

Stem-cell-based therapeutic approaches for Splenic Marginal Zone Lymphoma:

Hematopoietic stem cells for treatment of hematologic malignancies

Embryonic/Adult Cultured Cells Related to Splenic Marginal Zone Lymphoma:

Bone marrow-derived hematopoietic stem cells

Sources

Genetic Tests for Splenic Marginal Zone Lymphoma

Sources

Anatomical Context for Splenic Marginal Zone Lymphoma

Organs/tissues related to Splenic Marginal Zone Lymphoma:

FMA: Spleen Comprised Of B-Cells In Place Of White Pulp

MalaCards : Bone Marrow, B Cells, Spleen, Bone, T Cells, Liver, Myeloid

Sources

Publications for Splenic Marginal Zone Lymphoma

Articles related to Splenic Marginal Zone Lymphoma:

(show top 50) (show all 607)

#	Title	Authors	PMID	Year
1	Rituximab-induced severe acute thrombocytopenia in a patient with splenic marginal zone lymphoma. ⁶²	Kars TU...Demircioglu S	36458320	2022
2	Phenotype, Function, and Clinical Significance of CD26+ and CD161+Tregs in Splenic Marginal Zone Lymphoma. ⁶²	Tang X...Ansell SM	35686915	2022
3	Extranodal localization of non-Hodgkin's lymphoma in systemic sclerosis: A diagnostic challenge and review of the literature. ⁶²	Galoppini G...Govoni M	36211199	2022
4	Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency. ⁶²	Hanevelt J...de Vos Tot Nederveen Cappel WH	36241357	2022
5	Acquired angioedema: an unusual presentation of haematological malignancy. ⁶²	Farrugia D...Grech M	36123007	2022
6	Anti-CD20 rechallenge with ofatumumab in relapsed/refractory splenic marginal zone lymphoma: the MORE trial. ⁶²	Scarfo L...Ghia P	35613463	2022
7	Relevance of Additional Immunohistochemical Markers in the Differential Diagnosis of Small B-Cell Lymphomas: A Case-Control Study ⁶²	Kivrak H...Kuzu I	34619856	2022
8	Splenic Marginal Zone Lymphoma With Histological Transformation to Hodgkin's Lymphoma. ⁶²	Veeraballi S...Shaaban H	35967131	2022
9	Myeloid nuclear differentiation antigen: an aid in differentiating lymphoplasmacytic lymphoma and splenic marginal zone lymphoma in bone marrow biopsies at presentation. ⁶²	Righi S...Sabattini E	35339566	2022
10	Refractory Splenic Marginal Zone Lymphoma Responsive to Combination Venetoclax and Bortezomib (Velcade) (V2) Therapy. ⁶²	Roche KC...Aggarwal A	35735437	2022
11	Hairy lymphoproliferations: a case report and review of the literature ⁶²	Julien M...Lesesve JF	35796473	2022
12	Identification of a Cancer-Predisposing Germline POT1 p.Ile49Metfs*7 Variant by Targeted Sequencing of a Splenic Marginal Zone Lymphoma. ⁶²	Jajosky AN...Oduro KA	35456397	2022
13	Massive splenomegaly requiring differential diagnosis of hematologic malignancies. ⁶²	Tago M...Yamashita SI	35310319	2022
14	Immunohistochemistry for LEF1 and SOX11 adds diagnostic specificity in small B-cell lymphomas. ⁶²	Rangan A...Rech KL	35066013	2022
15	Genetic and phenotypic attributes of splenic marginal zone lymphoma. ⁶²	Bonfiglio F...Rossi D	34653238	2022
16	A new taxonomy for splenic marginal zone lymphoma. ⁶²	Deaglio S...Vaisitti T	35113148	2022
17	NOTCH signaling in the pathogenesis of splenic marginal zone lymphoma-opportunities for therapy. ⁶²	Alderuccio JP...Lossos IS	34586000	2022
18	Characteristics and survival outcomes of primary splenic cancers: A SEER population-based study. ⁶²	Lei Y...Liu M	35060511	2022
19	Risk-tailored treatment of splenic marginal zone lymphoma. ⁶²	Castelli R...Gidaro A	34407041	2022
20	[Warm autoimmune hemolytic anemia and IgM-monoclonal gammopathy following BNT162b2 COVID-19 vaccine in a patient with splenic marginal zone lymphoma]. ⁶²	Sogabe N...Hino M	36351643	2022
21	[Successful treatment of chronic idiopathic neutropenia in an elderly with ciclosporin]. ⁶²	Oshio M...Yamauchi T	35922943	2022
22	VS38 staining contributes to a novel gating strategy in flow cytometry for small B cell lymphoma, especially in lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. ⁶²	Mizuta S...Watanabe M	33682304	2022
23	Immunotherapy in indolent Non-Hodgkin's Lymphoma. ⁶²	Amhaz G...El-Cheikh J	35663281	2022
24	Clinico-biological features and outcome of patients with splenic marginal zone lymphoma with histological transformation. ⁶²	Bastidas-Mora G...Matutes E	34519021	2022
25	Repertoire of Rearranged Immunoglobulin Heavy Chain Genes in Russian Patients With B-Cell Lymphoproliferative Diseases. ⁶²	Biderman BV...Sudarikov AB	34384734	2021
26	A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma. ⁶²	Yilmaz E...Aggarwal A	34940070	2021
27	CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen. ⁶²	Schmieg JJ...Auerbach A	34940069	2021
28	Cold agglutinin syndrome secondary to splenic marginal zone lymphoma: a case report. ⁶²	Portich JP...Franz JPM	34836848	2021
29	Clinical Characteristics, Treatment and Evolution of Splenic and Nodal Marginal Zone Lymphomas-Retrospective and Multicentric Analysis of Portuguese Centers. ⁶²	Rodrigues CD...Coutinho R	34326035	2021
30	How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen. ⁶²	Cabecadas J...Gomes da Silva M	34898558	2021
31	The effect of surgery on primary splenic lymphoma: A study based on SEER database. ⁶²	Pan X...Zhao J	34547191	2021
32	The relative expression levels of CD148 and CD180 on clonal B cells and CD148/CD180 median fluorescence intensity ratios are useful in the characterization of mature B cell lymphoid neoplasms infiltrating blood and bone marrow - Results from a single centre pilot study. ⁶²	Gautam A...Varma N	33455071	2021
33	Acquired C1-inhibitor deficiency due to splenic marginal zone lymhoma: Case Report. ⁶²	Verschueren J...Gheysens O	32228367	2021
34	Hepatic Infiltration by Splenic Marginal Zone Lymphoma in a Patient With Cured Hepatitis C. ⁶²	Silva L...Lopes Cacola R	34786250	2021
35	New Insights into the Biology and Diagnosis of Splenic Marginal Zone Lymphomas. ⁶²	Donzel M...Traverse-Glehen A	34590593	2021
36	Frequency of MYD88 L256P mutation and its correlation with clinico-hematological profile in mature B-cell neoplasm. ⁶²	Shekhar R...Malhotra P	33217360	2021
37	A Rare Case of Factor VII Inhibitor in a Patient Presenting with Primary Splenic Marginal Zone Lymphoma. ⁶²	Naismith K...Hamm C	34487513	2021
38	Undetectable minimal residual disease is an independent prognostic factor in splenic marginal zone lymphoma. ⁶²	Lyu R...Yi S	34328213	2021
39	Lymphocyte Aggregation in Low-Grade B-Cell Lymphoma. ⁶²	Walradth EA	33900388	2021
40	Superior outcome for splenectomised patients in a population-based study of splenic marginal zone lymphoma in Sweden. ⁶²	Sima A...Amini RM	34109612	2021
41	Chronic kidney disease linked to SARS-CoV-2 infection: a case report. ⁶²	Tarris G...Martin L	34376184	2021
42	Splenic marginal zone lymphoma with increased prolymphocytes. ⁶²	Cai J...Qing X	34081119	2021
43	Radiologically guided percutaneous core needle biopsy of the spleen: a reliable and safe diagnostic procedure for neoplastic and reactive conditions. ⁶²	Sangiorgio VFI...Calaminici M	33393079	2021
44	Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy: A case report and review of the literature. ⁶²	Ababou M...Doghmi K	31306619	2021
45	Atypical CD5 and CD10 coexpression in a splenic marginal zone lymphoma. ⁶²	Gimenez De Mestral S...Suner L	35845281	2021
46	[Rituximab-induced acute thrombocytopenia in a patient with splenic marginal zone lymphoma: a case report and literature review]. ⁶²	Huang AJ...Wang JM	33979980	2021
47	Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature. ⁶²	Evans MG...Nichter LS	33898650	2021
48	Epidemiology of Marginal Zone Lymphoma. ⁶²	Cerhan JR...Habermann TM	33829216	2021
49	Peripheral blood morphology review and diagnostic proficiency evaluation by a new Spanish EQAS during the period 2011-2019. ⁶²	Alcaraz-Quiles J...Merino A	32870604	2021
50	Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma. ⁶²	Thongtan T...Nugent K	34219945	2021

Sources

Genes for Splenic Marginal Zone Lymphoma

Genes related to Splenic Marginal Zone Lymphoma (0 elite genes):

(show all 25)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	PAX5	Paired Box 5	Protein Coding	13.76	DISEASES inferred ¹⁴ GeneCards inferred via : Functions (show sections)
2	IGHV4-38-2	Immunoglobulin Heavy Variable 4-38-2	Protein Coding	7.59	DISEASES inferred ¹⁴
3	U2AF1	U2 Small Nuclear RNA Auxiliary Factor 1	Protein Coding	7.56	DISEASES inferred ¹⁴ ¹⁴
4	CD5	CD5 Molecule	Protein Coding	7.46	DISEASES inferred ¹⁴
5	MME	Membrane Metalloendopeptidase	Protein Coding	6.96	DISEASES inferred ¹⁴
6	ITGAE	Integrin Subunit Alpha E	Protein Coding	6.89	DISEASES inferred ¹⁴
7	NOTCH2	Notch Receptor 2	Protein Coding	6.87	DISEASES inferred ¹⁴
8	CD22	CD22 Molecule	Protein Coding	6.83	DISEASES inferred ¹⁴
9	BCL6	BCL6 Transcription Repressor	Protein Coding	6.76	DISEASES inferred ¹⁴
10	CD19	CD19 Molecule	Protein Coding	6.65	DISEASES inferred ¹⁴
11	CD79A	CD79a Molecule	Protein Coding	6.59	DISEASES inferred ¹⁴
12	CD79B	CD79b Molecule	Protein Coding	6.47	DISEASES inferred ¹⁴
13	ITGAX	Integrin Subunit Alpha X	Protein Coding	6.41	DISEASES inferred ¹⁴
14	KLF2	KLF Transcription Factor 2	Protein Coding	6.37	DISEASES inferred ¹⁴
15	MYD88	MYD88 Innate Immune Signal Transduction Adaptor	Protein Coding	6.34	DISEASES inferred ¹⁴
16	SPN	Sialophorin	Protein Coding	6.32	DISEASES inferred ¹⁴
17	CCR6	C-C Motif Chemokine Receptor 6	Protein Coding	6.31	DISEASES inferred ¹⁴ ¹⁴
18	CARD11	Caspase Recruitment Domain Family Member 11	Protein Coding	6.27	DISEASES inferred ¹⁴
19	IL3RA	Interleukin 3 Receptor Subunit Alpha	Protein Coding	6.27	DISEASES inferred ¹⁴
20	TRAF3	TNF Receptor Associated Factor 3	Protein Coding	6.26	DISEASES inferred ¹⁴
21	BIRC3	Baculoviral IAP Repeat Containing 3	Protein Coding	6.12	DISEASES inferred ¹⁴
22	CCND1	Cyclin D1	Protein Coding	6.11	DISEASES inferred ¹⁴
23	TNFAIP3	TNF Alpha Induced Protein 3	Protein Coding	6.11	DISEASES inferred ¹⁴
24	ANXA1	Annexin A1	Protein Coding	6.05	DISEASES inferred ¹⁴
25	MALT1	MALT1 Paracaspase	Protein Coding	6	DISEASES inferred ¹⁴

Sources

Variations for Splenic Marginal Zone Lymphoma

Copy number variations for Splenic Marginal Zone Lymphoma from CNVD:

⁶ (show top 50) (show all 56)

#	CNVD ID	Chromosome	Start	End	Type	Gene Symbol	CNVD Disease
1	31168	1	25800000	154800000	Duplication		Splenic marginal zone lymphoma
2	38774	10	104900000	119100000	Deletion		Splenic marginal zone lymphoma
3	56497	11	59700000	61400000	Duplication		Splenic marginal zone lymphoma
4	69123	12	53100000	55200000	Gain	GLI1	Splenic marginal zone lymphoma
5	78495	13	52200000	109100000	Gain		Splenic marginal zone lymphoma
6	86225	14	55800000	78400000	Loss		Splenic marginal zone lymphoma
7	91971	15	37900000	47600000	Deletion		Splenic marginal zone lymphoma
8	97450	16	10300000	16700000	Gain		Splenic marginal zone lymphoma
9	106744	17	1	11200000	Deletion		Splenic marginal zone lymphoma
10	106745	17	1	11200000	Deletion		Splenic marginal zone lymphoma
11	108750	17	22200000	78774742	Duplication		Splenic marginal zone lymphoma
12	119209	18	1	76117153	Duplication		Splenic marginal zone lymphoma
13	120285	18	23300000	31000000	Deletion		Splenic marginal zone lymphoma
14	134527	2	102400000	159600000	Deletion		Splenic marginal zone lymphoma
15	155734	20	57900000	62435964	Duplication		Splenic marginal zone lymphoma
16	160287	22	11800000	49691432	Duplication		Splenic marginal zone lymphoma
17	166594	3	104400000	107800000	Deletion		Splenic marginal zone lymphoma
18	173065	3	189400000	193800000	Duplication		Splenic marginal zone lymphoma
19	177287	3	5500000	8700000	Deletion		Splenic marginal zone lymphoma
20	178512	3	74200000	83700000	Duplication		Splenic marginal zone lymphoma
21	181981	4	131300000	151000000	Deletion		Splenic marginal zone lymphoma
22	190152	4	84000000	161500000	Deletion		Splenic marginal zone lymphoma
23	190418	4	88200000	94000000	Duplication		Splenic marginal zone lymphoma
24	201328	5	68400000	73300000	Duplication		Splenic marginal zone lymphoma
25	207408	6	155600000	160900000	Duplication		Splenic marginal zone lymphoma
26	209385	6	26100000	45200000	Duplication		Splenic marginal zone lymphoma
27	212692	6	40600000	45200000	Duplication		Splenic marginal zone lymphoma
28	217433	7	101175494	101390682	Deletion	CUX1	Splenic marginal zone lymphoma
29	217434	12	109956211	110272739	Deletion	CUX2	Splenic marginal zone lymphoma
30	217435	7	101175494	101390682	Deletion	MUC12	Splenic marginal zone lymphoma
31	217436	7	101175494	101390682	Deletion	MUC3A	Splenic marginal zone lymphoma
32	218058	7	107200000	147500000	Deletion		Splenic marginal zone lymphoma
33	219020	7	120900000	123600000	Loss	POT1	Splenic marginal zone lymphoma
34	219788	7	130078078	130270796	Deletion	AGK	Splenic marginal zone lymphoma
35	219789	7	130078078	130270796	Deletion	BPGM	Splenic marginal zone lymphoma
36	219790	7	130078078	130270796	Deletion	CALD1	Splenic marginal zone lymphoma
37	219791	7	130078078	130270796	Deletion	CNTNAP2	Splenic marginal zone lymphoma
38	219792	7	130078078	130270796	Deletion	FOXP2	Splenic marginal zone lymphoma
39	219793	7	130078078	130270796	Deletion	GRM8	Splenic marginal zone lymphoma
40	219794	7	130078078	130270796	Deletion	IMMP2L	Splenic marginal zone lymphoma
41	219795	7	130078078	130270796	Deletion	KEL	Splenic marginal zone lymphoma
42	219796	7	130078078	130270796	Deletion	MET	Splenic marginal zone lymphoma
43	219797	7	130078078	130270796	Deletion		Splenic marginal zone lymphoma
44	219798	7	130078078	130270796	Deletion	PTN	Splenic marginal zone lymphoma
45	219799	7	130078078	130270796	Deletion	PTPRZ1	Splenic marginal zone lymphoma
46	219800	7	130078078	130270796	Deletion	ST7	Splenic marginal zone lymphoma
47	219801	7	130078078	130270796	Deletion	SVOPL	Splenic marginal zone lymphoma
48	219802	7	130078078	130270796	Deletion	TES	Splenic marginal zone lymphoma
49	219803	7	130078078	130270796	Deletion	THAP5	Splenic marginal zone lymphoma
50	219804	7	130078078	130270796	Deletion	TPK1	Splenic marginal zone lymphoma

Sources

Expression for Splenic Marginal Zone Lymphoma

Search GEO for disease gene expression data for Splenic Marginal Zone Lymphoma.

Sources

Pathways for Splenic Marginal Zone Lymphoma

Pathways related to Splenic Marginal Zone Lymphoma according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	Innate Immune System ⁶⁶ Show member pathways Immune System ⁶⁶ Neutrophil degranulation ⁶⁶	13.76	TRAF3 MYD88 MME ITGAX IL3RA CD79B
2	NF-kappaB Signaling ³	12.38	BCL6 CARD11 CD19 CD79A MYD88 PAX5
3	Immune response Fc epsilon RI pathway ²² Show member pathways Fc-epsilon receptor I signaling in mast cells ⁶¹ Immune response BCR pathway ²²	12.13	CD79B CD79A CD22 CD19 BCL6
4	Dendritic Cells Developmental Lineage Pathway ⁶⁵	11.6	SPN MME ITGAX ITGAE IL3RA CD19
5	Innate Lymphoid Cells Differentiation ⁶⁵	11.54	NOTCH2 ITGAE CCR6
6	Extrafollicular B cell activation by SARS-CoV-2 ⁷⁴	11.48	PAX5 ITGAX CD79A CD22 CD19 BCL6
7	Hematopoietic Stem Cells and Lineage-specific Markers ⁶⁵	11.39	SPN NOTCH2 MME ITGAX IL3RA CD79B
8	Antigen activates B Cell Receptor (BCR) leading to generation of second messengers ⁶⁶ Show member pathways B-Cell Receptor Complex ⁶⁴ CD22 mediated BCR regulation ⁶⁶	11.26	CD79B CD79A CD22 CD19
9	BCR signaling pathway ⁶¹	11.25	CD79B CD79A CD22 CD19 CARD11 BCL6

Sources

GO Terms for Splenic Marginal Zone Lymphoma

Cellular components related to Splenic Marginal Zone Lymphoma according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	membrane	GO:0016021	10.42	CCR6 CD19 CD22 CD5 CD79A CD79B
2	membrane	GO:0016020	10.42	CCR6 CD19 CD22 CD5 CD79A CD79B
3	plasma membrane	GO:0005887	10.03	CCR6 CD19 CD22 CD5 CD79B MME
4	plasma membrane	GO:0005886	10.03	CCR6 CD19 CD22 CD5 CD79B MME
5	external side of plasma membrane	GO:0009897	9.64	SPN ITGAX ITGAE IL3RA IGHV4-38-2 CD79B
6	B cell receptor complex	GO:0019815	9.46	CD79B CD79A

Biological processes related to Splenic Marginal Zone Lymphoma according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	B cell receptor signaling pathway	GO:0050853	9.87	IGHV4-38-2 CD79B CD79A CD19
2	B cell proliferation	GO:0042100	9.85	CD79A CARD11 BCL6
3	regulation of T cell migration	GO:2000404	9.71	SPN CCR6
4	immune system process	GO:0002376	9.7	BCL6 CARD11 CD19 CD79A CD79B MYD88
5	Toll signaling pathway	GO:0008063	9.56	TRAF3 MYD88
6	regulation of immune response	GO:0050776	9.5	SPN CD22 CARD11 BCL6
7	B cell differentiation	GO:0030183	9.28	PAX5 CD79B CD79A CARD11 BCL6