SMZL
MCID: SPL004
MIFTS: 48

Splenic Marginal Zone Lymphoma (SMZL)

Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases
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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:

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
UMLS 71 C0349632

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...

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:



Diseases related to Splenic Marginal Zone Lymphoma

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

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
1
Chlorambucil Approved Phase 3 305-03-3 2708
2
Ondansetron Approved, Withdrawn Phase 2 99614-02-5 4595
3
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 38904
4
Melphalan Approved Phase 2 148-82-3 4053 460612
5
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
6
Vorinostat Approved, Investigational Phase 1, Phase 2 149647-78-9 5311
7
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
8
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
9
Iodine Approved, Investigational Phase 2 7553-56-2 807
10
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
11
Clotrimazole Approved, Vet_approved Phase 1, Phase 2 23593-75-1 2812
12
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
13
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
14
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
15
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
16
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
17
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
18
Panobinostat Approved, Investigational Phase 1, Phase 2 404950-80-7 6918837
19
Everolimus Approved Phase 1, Phase 2 159351-69-6 70789204 6442177
20
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030
21
Alemtuzumab Approved, Investigational Phase 2 216503-57-0
22
Levoleucovorin Approved, Experimental, Investigational Phase 2 68538-85-2, 58-05-9, 73951-54-9 149436 6006
23
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 4112 126941
24
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
25
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
26
Idelalisib Approved Phase 2 870281-82-6 11654566 11625818
27
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
28
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
29
Lenograstim Approved, Investigational Phase 1, Phase 2 135968-09-1
30
Busulfan Approved, Investigational Phase 2 55-98-1 2478
31
Ipilimumab Approved Phase 1, Phase 2 477202-00-9
32
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
33
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
34
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
35
Oprelvekin Approved, Investigational Phase 1, Phase 2 145941-26-0
36
Iron Approved Phase 2 7439-89-6 29936
37
Deferasirox Approved, Investigational Phase 2 201530-41-8 214348 5493381
38
Brentuximab vedotin Approved, Investigational Phase 1, Phase 2 914088-09-8
39
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 2 63-91-2, 673-06-3 6140 71567
40
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
41
Cadexomer iodine Experimental Phase 2 94820-09-4
42
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7 4897
43
Palifosfamide Investigational Phase 1, Phase 2 31645-39-3 100427
44
Tipifarnib Investigational Phase 2 192185-72-1 159324
45
Bryostatin 1 Investigational Phase 2 83314-01-6
46
Veliparib Investigational Phase 1, Phase 2 912444-00-9 11960529
47
Alvocidib Experimental, Investigational Phase 1, Phase 2 131740-09-5, 146426-40-6 5466794 5287969
48
Fenretinide Investigational Phase 1, Phase 2 65646-68-6 5288209
49 Antipsychotic Agents Phase 2
50 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:


LifeMap Discovery
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

Genetic Tests for Splenic Marginal Zone Lymphoma

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

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
36458320 2022
2
Phenotype, Function, and Clinical Significance of CD26+ and CD161+Tregs in Splenic Marginal Zone Lymphoma. 62
35686915 2022
3
Extranodal localization of non-Hodgkin's lymphoma in systemic sclerosis: A diagnostic challenge and review of the literature. 62
36211199 2022
4
Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency. 62
36241357 2022
5
Acquired angioedema: an unusual presentation of haematological malignancy. 62
36123007 2022
6
Anti-CD20 rechallenge with ofatumumab in relapsed/refractory splenic marginal zone lymphoma: the MORE trial. 62
35613463 2022
7
Relevance of Additional Immunohistochemical Markers in the Differential Diagnosis of Small B-Cell Lymphomas: A Case-Control Study 62
34619856 2022
8
Splenic Marginal Zone Lymphoma With Histological Transformation to Hodgkin's Lymphoma. 62
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
35339566 2022
10
Refractory Splenic Marginal Zone Lymphoma Responsive to Combination Venetoclax and Bortezomib (Velcade) (V2) Therapy. 62
35735437 2022
11
Hairy lymphoproliferations: a case report and review of the literature 62
35796473 2022
12
Identification of a Cancer-Predisposing Germline POT1 p.Ile49Metfs*7 Variant by Targeted Sequencing of a Splenic Marginal Zone Lymphoma. 62
35456397 2022
13
Massive splenomegaly requiring differential diagnosis of hematologic malignancies. 62
35310319 2022
14
Immunohistochemistry for LEF1 and SOX11 adds diagnostic specificity in small B-cell lymphomas. 62
35066013 2022
15
Genetic and phenotypic attributes of splenic marginal zone lymphoma. 62
34653238 2022
16
A new taxonomy for splenic marginal zone lymphoma. 62
35113148 2022
17
NOTCH signaling in the pathogenesis of splenic marginal zone lymphoma-opportunities for therapy. 62
34586000 2022
18
Characteristics and survival outcomes of primary splenic cancers: A SEER population-based study. 62
35060511 2022
19
Risk-tailored treatment of splenic marginal zone lymphoma. 62
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
36351643 2022
21
[Successful treatment of chronic idiopathic neutropenia in an elderly with ciclosporin]. 62
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
33682304 2022
23
Immunotherapy in indolent Non-Hodgkin's Lymphoma. 62
35663281 2022
24
Clinico-biological features and outcome of patients with splenic marginal zone lymphoma with histological transformation. 62
34519021 2022
25
Repertoire of Rearranged Immunoglobulin Heavy Chain Genes in Russian Patients With B-Cell Lymphoproliferative Diseases. 62
34384734 2021
26
A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma. 62
34940070 2021
27
CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen. 62
34940069 2021
28
Cold agglutinin syndrome secondary to splenic marginal zone lymphoma: a case report. 62
34836848 2021
29
Clinical Characteristics, Treatment and Evolution of Splenic and Nodal Marginal Zone Lymphomas-Retrospective and Multicentric Analysis of Portuguese Centers. 62
34326035 2021
30
How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen. 62
34898558 2021
31
The effect of surgery on primary splenic lymphoma: A study based on SEER database. 62
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
33455071 2021
33
Acquired C1-inhibitor deficiency due to splenic marginal zone lymhoma: Case Report. 62
32228367 2021
34
Hepatic Infiltration by Splenic Marginal Zone Lymphoma in a Patient With Cured Hepatitis C. 62
34786250 2021
35
New Insights into the Biology and Diagnosis of Splenic Marginal Zone Lymphomas. 62
34590593 2021
36
Frequency of MYD88 L256P mutation and its correlation with clinico-hematological profile in mature B-cell neoplasm. 62
33217360 2021
37
A Rare Case of Factor VII Inhibitor in a Patient Presenting with Primary Splenic Marginal Zone Lymphoma. 62
34487513 2021
38
Undetectable minimal residual disease is an independent prognostic factor in splenic marginal zone lymphoma. 62
34328213 2021
39
Lymphocyte Aggregation in Low-Grade B-Cell Lymphoma. 62
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40
Superior outcome for splenectomised patients in a population-based study of splenic marginal zone lymphoma in Sweden. 62
34109612 2021
41
Chronic kidney disease linked to SARS-CoV-2 infection: a case report. 62
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42
Splenic marginal zone lymphoma with increased prolymphocytes. 62
34081119 2021
43
Radiologically guided percutaneous core needle biopsy of the spleen: a reliable and safe diagnostic procedure for neoplastic and reactive conditions. 62
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
31306619 2021
45
Atypical CD5 and CD10 coexpression in a splenic marginal zone lymphoma. 62
35845281 2021
46
[Rituximab-induced acute thrombocytopenia in a patient with splenic marginal zone lymphoma: a case report and literature review]. 62
33979980 2021
47
Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature. 62
33898650 2021
48
Epidemiology of Marginal Zone Lymphoma. 62
33829216 2021
49
Peripheral blood morphology review and diagnostic proficiency evaluation by a new Spanish EQAS during the period 2011-2019. 62
32870604 2021
50
Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma. 62
34219945 2021

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

Expression for Splenic Marginal Zone Lymphoma

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

Pathways for Splenic Marginal Zone Lymphoma

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

Sources for Splenic Marginal Zone Lymphoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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