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SMZL
MCID: SPL004
MIFTS: 51

Splenic Marginal Zone Lymphoma (SMZL)

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Splenic Marginal Zone Lymphoma

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MalaCards integrated aliases for Splenic Marginal Zone Lymphoma:

Name: Splenic Marginal Zone Lymphoma 12 58 15 37 17
Splenic Marginal Zone B-Cell Lymphoma 71
Smzl 58

Characteristics:

Orphanet epidemiological data:

58
splenic marginal zone lymphoma
Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Blood diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:0050750
ICD10 via Orphanet 33 C83.0
UMLS via Orphanet 72 C0349632
Orphanet 58 ORPHA86854
UMLS 71 C0349632
Sources

Summaries for Splenic Marginal Zone Lymphoma

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Disease Ontology : 12 A marginal zone B-cell lymphocyte located in the spleen comprised of B-cells in place of white pulp.

MalaCards based summary : Splenic Marginal Zone Lymphoma, also known as splenic marginal zone b-cell lymphoma, is related to nodal marginal zone b-cell lymphoma and marginal zone b-cell lymphoma. An important gene associated with Splenic Marginal Zone Lymphoma is NOTCH2 (Notch Receptor 2), and among its related pathways/superpathways are TNFR1 Pathway and Toll-like Receptor Signaling Pathway. The drugs Thiotepa and Pixantrone have been mentioned in the context of this disorder. Affiliated tissues include spleen comprised of b-cells in place of white pulp, b cells and bone, and related phenotypes are hematopoietic system and immune system

Wikipedia : 74 Splenic marginal zone lymphoma (SMZL) is a type of cancer (specifically a lymphoma) made up of B-cells... more...

Sources

Related Diseases for Splenic Marginal Zone Lymphoma

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

(show top 50) (show all 187)
# Related Disease Score Top Affiliating Genes
1 nodal marginal zone b-cell lymphoma 31.3 MALT1 BCL6
2 marginal zone b-cell lymphoma 31.2 SDC1 PAX5 MALT1 FOXP1 CD5 BTK
3 lymphoma, hodgkin, classic 31.1 TRAF3 PAX5 CD40 BCL6
4 nodal marginal zone lymphoma 31.1 NOTCH2 CD5 BCL6
5 composite lymphoma 30.9 PAX5 CD5 BCL6
6 macroglobulinemia 30.7 SYK SDC1 PAX5 BTK
7 splenic disease 30.5 ITGAE CD5 BCL6
8 lymphoplasmacytic lymphoma 30.5 PAX5 CD5 CD40 BTK BIRC3
9 lymphoma 30.4 PAX5 NFKB2 MALT1 CDK6 CD5 CD40
10 cll/sll 30.4 PAX5 CD5 BTK BCL6
11 follicular lymphoma 30.1 SYK PAX5 CD5 CD40 BCL6 AICDA
12 waldenstroem's macroglobulinemia 29.8 TRAF3 SYK SDC1 CD5 BTK
13 immune deficiency disease 29.7 CD5 CD40 BTK BCL6 AICDA
14 mature b-cell neoplasm 29.6 SDC1 PAX5 CD5 CD40 BCL6 AICDA
15 burkitt lymphoma 29.5 PAX5 CDK6 CD40 BIRC3 BCL6 AICDA
16 mantle cell lymphoma 29.4 SYK PAX5 MALT1 CDK6 CD5 CD40
17 b-cell lymphoma 29.3 SYK PAX5 NFKB2 MALT1 FOXP1 CD5
18 lymphoma, non-hodgkin, familial 28.9 SYK SDC1 PAX5 MALT1 CDK6 CD5
19 lymphoma, mucosa-associated lymphoid type 28.9 SDC1 PAX5 MALT1 FOXP1 CD5 CD40
20 diffuse large b-cell lymphoma 28.7 SYK SDC1 PAX5 MALT1 FOXP1 CD5
21 myeloma, multiple 28.6 TRAF3 SDC1 PAX5 NFKB2 CDK6 CD5
22 leukemia, chronic lymphocytic 28.6 SYK SDC1 PAX5 NOTCH2 ITGAE CDK6
23 splenomegaly 10.9
24 hemolytic anemia 10.6
25 lymphoproliferative syndrome 10.5
26 anemia, autoimmune hemolytic 10.5
27 hepatitis c virus 10.5
28 bladder lymphoma 10.5 CD5 BCL6
29 nodular lymphocyte predominant hodgkin lymphoma 10.5 PAX5 BCL6
30 anemia, x-linked, with or without neutropenia and/or platelet abnormalities 10.5
31 deficiency anemia 10.5
32 paranasal sinus lymphoma 10.5 PAX5 BCL6
33 prostate lymphoma 10.4 CD5 BCL6
34 epiglottis neoplasm 10.4 CD5 BCL6
35 hairy cell leukemia 10.4
36 indolent b cell lymphoma 10.4
37 hodgkin's lymphoma, nodular sclerosis 10.4 PAX5 BCL6
38 intravascular large b-cell lymphoma 10.4 CD5 BCL6
39 monoclonal paraproteinemia 10.4 SDC1 PAX5
40 colon lymphoma 10.4 CD5 BCL6
41 appendix lymphoma 10.4 CD5 BCL6
42 gray zone lymphoma 10.4 PAX5 BCL6
43 orbit lymphoma 10.4 MALT1 FOXP1
44 pancytopenia 10.4
45 thrombocytopenia 10.4
46 hepatitis c 10.4
47 chromosomal triplication 10.4
48 b-cell non-hodgkin lymphoma 10.4
49 eye lymphoma 10.4 CD5 BCL6
50 bone lymphoma 10.4 PAX5 CD5 BCL6

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



Diseases related to Splenic Marginal Zone Lymphoma
Sources

Symptoms & Phenotypes for Splenic Marginal Zone Lymphoma

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MGI Mouse Phenotypes related to Splenic Marginal Zone Lymphoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.36 AICDA BCL6 BIRC3 BTK CD40 CD5
2 immune system MP:0005387 10.3 AICDA BCL6 BIRC3 BTK CD40 CD5
3 cellular MP:0005384 10.26 AICDA BCL6 BIRC3 BTK CDK6 CREBBP
4 homeostasis/metabolism MP:0005376 10.22 AICDA BCL6 BIRC3 BTK CD40 CREBBP
5 endocrine/exocrine gland MP:0005379 10.11 BCL6 CD40 CDK6 CREBBP NFKB2 NOTCH2
6 mortality/aging MP:0010768 10.1 AICDA BCL6 BIRC3 BTK CDK6 CREBBP
7 liver/biliary system MP:0005370 9.92 BCL6 CDK6 CREBBP KLF2 NFKB2 NOTCH2
8 neoplasm MP:0002006 9.7 AICDA BTK CDK6 CREBBP NOTCH2 PAX5
9 no phenotypic analysis MP:0003012 9.5 AICDA BCL6 CD5 KLF2 NOTCH2 SYK
10 normal MP:0002873 9.28 AICDA BCL6 BIRC3 CD40 CD5 CREBBP
Sources

Drugs & Therapeutics for Splenic Marginal Zone Lymphoma

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

(show top 50) (show all 296)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Thiotepa Approved, Investigational Phase 4 52-24-4 5453
2
Pixantrone Approved, Investigational Phase 3 144510-96-3
3
Idarubicin Approved Phase 3 58957-92-9 42890
4 Orange Approved Phase 3
5
Cefepime Approved, Investigational Phase 3 88040-23-7 5479537
6
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
7
Chlorambucil Approved Phase 3 305-03-3 2708
8
Carboplatin Approved Phase 2, Phase 3 41575-94-4 10339178 38904 498142
9
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
10
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
11
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
12
Iron Approved, Experimental Phase 3 15438-31-0, 7439-89-6 27284 23925
13
Itraconazole Approved, Investigational Phase 3 84625-61-6 55283
14
Dalteparin Approved Phase 3 9005-49-6
15
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
16
Levofloxacin Approved, Investigational Phase 3 100986-85-4 149096
17
Ofloxacin Approved Phase 3 82419-36-1 4583
18
Captopril Approved Phase 3 62571-86-2 44093
19
Fluconazole Approved, Investigational Phase 3 86386-73-4 3365
20
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
21
Ribavirin Approved Phase 3 36791-04-5 37542
22
Palivizumab Approved, Investigational Phase 3 188039-54-5
23
Acyclovir Approved Phase 3 59277-89-3 2022
24
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
25
Darbepoetin alfa Approved, Investigational Phase 3 11096-26-7, 209810-58-2
26
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 2826718 468682
27
Tazobactam Approved Phase 3 89786-04-9 123630
28
Vancomycin Approved Phase 3 1404-90-6 14969 441141
29
Piperacillin Approved Phase 3 66258-76-2 43672
30
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
31
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
32
Norgestimate Approved, Investigational Phase 3 35189-28-7 6540478
33
Moxifloxacin Approved, Investigational Phase 3 354812-41-2, 151096-09-2 152946
34
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
35
Polyestradiol phosphate Approved Phase 3 28014-46-2
36
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
37
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
38
Epratuzumab Investigational Phase 2, Phase 3 205923-57-5
39 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
40 Ferric Oxide, Saccharated Phase 3
41 Dextrans Phase 3
42 Plasma Substitutes Phase 3
43 Iron-Dextran Complex Phase 3
44 Blood Substitutes Phase 3
45 Anesthetics, Dissociative Phase 3
46 Adjuvants, Immunologic Phase 3
47 Antineoplastic Agents, Hormonal Phase 3
48 Tubulin Modulators Phase 3
49 Antimitotic Agents Phase 3
50 interferons Phase 3

Interventional clinical trials:

(show top 50) (show all 369)
# Name Status NCT ID Phase Drugs
1 Evaluation of Allogeneic Marrow Transplants Depleted of T-Cells by CD34+ Selection in Patients Undergoing Transplantation With an Unrelated Matched or 1 Antigen Mismatched Donor or a 1 Antigen Mismatched Related Donor Completed NCT00003398 Phase 4 cyclophosphamide;thiotepa
2 A European Randomized Multicenter Study of Interferon Alfa-2b Versus No Treatment After Intensive Therapy and Autologous Hematopoietic Stem Cell Transplantation for Relapsing Lymphoma Patients (Non-Hodgkin Lymphomas and Hodgkin's Disease) Unknown status NCT00003924 Phase 3
3 A Randomized Phase III Trial Comparing the Combination of Fludarabine, BBR 2778 (Pixantrone) and Rituximab (FP-R) With the Combination of Fludarabine and Rituximab (F-R) in the Treatment of Patients With Relapsed or Refractory Indolent Non-Hodgkin's Lymphoma Unknown status NCT00551239 Phase 3 fludarabine phosphate;pixantrone dimaleate
4 Randomised Controlled Trial of CID (Chlorambucil, Idarubicin, Dexamethasone) Versus CD (Chlorambucil, Dexamethasone) for Induction of Remission in Low Grade Non-Hodgkin's Lymphoma (Kiel Classification) Followed by Randomised Controlled Assessment of Standard Dose Interferon Versus Low Dose Interferon Versus No Further Therapy as Maintenance Treatment After Remission Induction Unknown status NCT00003639 Phase 3 chlorambucil;dexamethasone;idarubicin
5 Randomized Controlled Study of Iron Supplementation to Support the Response to Recombinant Human Erythropoietin for the Treatment of Chemotherapy-Induced Anaemia Unknown status NCT00482716 Phase 3
6 The Utility of LymphoScan Imaging in the Dectection of Residual Tumor After Chemotherapy and/or Radiotherapy in Patients With Non-Hodgkin's Lymphoma Unknown status NCT00003338 Phase 2, Phase 3
7 The Utility of LymphoScan Imaging in the Localization and Staging of Patients With Non-Hodgkin's Lymphoma Unknown status NCT00003337 Phase 3
8 A Randomized Phase III Study to Assess Intensification of the Conditioning Regimen for Allogenic Stem Cell Transplantation (ALLO-SCT) for Leukemia or Myelodysplastic Syndrome With a High Risk of Relapse Unknown status NCT00002989 Phase 3 busulfan;cyclophosphamide;idarubicin;melphalan
9 Randomised Study Comparing 6 And 8 Cycles Of Chemotherapy With CHOP ( Cyclophosphamide, Doxorubicin, Vincristine And Prednisone) At 14-Day Intervals (CHOP-14), Both With Or Without The Monoclonal Anti-CD20 Antibody Rituximab In Patients Aged 61 To 80 Years With Aggressive Non-Hodgkin's Lymphoma Unknown status NCT00052936 Phase 3 CHOP regimen;cyclophosphamide;doxorubicin hydrochloride;prednisone;vincristine sulfate
10 A Prospective Randomised Phase III Trial of Early Hospital Discharge Versus Standard Inpatient Management of Cancer Patients With Low-Risk Febrile Neutropenia Receiving Oral Antibiotics. Oral Antibiotics for Neutropenic Sepsis Giving Early Hospital Discharge [ORANGE] Unknown status NCT00445497 Phase 3 amoxicillin-clavulanate potassium;ciprofloxacin
11 Multicenter, Double-Blind, Randomized Study to Compare the Safety and Efficacy of Levofloxacin With That of Cefepime in the Treatment of Fever and Neutropenia - Phase IIIB Unknown status NCT00020865 Phase 3 cefepime hydrochloride;levofloxacin
12 A Randomized Double-Blind Controlled Trial of Ketamine Versus Placebo in Conjunction With Best Pain Management in Neuropathic Pain in Cancer Patients Unknown status NCT01316744 Phase 3 ketamine hydrochloride
13 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
14 A Randomised Trial of Chlorambucil Versus Fludarabine as Initial Therapy of Waldenström's Macroglobulinaemia and Splenic Lymphoma With Villous Lymphocytes Completed NCT00608374 Phase 3 chlorambucil;fludarabine phosphate
15 Randomized Trial of Autologous GVHD for Refractory Lymphoma Completed NCT00003414 Phase 3 busulfan;cyclophosphamide;cyclosporine
16 A Phase III Randomized Trial of CHOP Chemotherapy Plus Rituxan (IDEC-C2B8) Versus CHOP Chemotherapy Alone for Newly Diagnosed, Previously Untreated, Aggressive Non-Hodgkin's Lymphoma Completed NCT00004112 Phase 3 CHOP regimen;cyclophosphamide;doxorubicin hydrochloride;prednisone;vincristine sulfate
17 A Double-Blind Study Of Nutritional Intervention For The Treatment Of Cancer Cachexia Using Juven® Nutritional Supplement Completed NCT00053053 Phase 3
18 Randomized Placebo Controlled Double Blind Study of Restasis Versus Placebo in Primary Prevention of Ocular GVHD After Allogeneic Stem Cell Transplantation Completed NCT00755040 Phase 3 cyclosporine ophthalmic emulsion
19 A Randomized Multicentre Study Comparing G-CSF Mobilized Peripheral Blood and G-CSF Stimulated Bone Marrow in Patients Undergoing Matched Sibling Transplantation for Hematologic Malignancies Completed NCT00438958 Phase 3
20 Phase III, Randomized, Double-Blind, Placebo-Controlled Crossover Trial of Ondansetron in the Control of Chronic Nausea and Vomiting Not Due to Antineoplastic Therapy in Patients With Advanced Cancer Completed NCT00006348 Phase 3 ondansetron
21 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
22 A Randomized, Prospective Double-Blind, Placebo-Controlled Trial of Prophylactic Oral Levofloxacin Following Chemotherapy for Lymphoma and Solid Tumors Completed NCT00005590 Phase 3 levofloxacin
23 Phase III Study of Captopril in Patients Undergoing Autologous Bone Marrow/Stem Cell Transplantation Completed NCT00004230 Phase 3 captopril;cyclophosphamide
24 A Randomized, Comparative Study of Itraconazole Versus Fluconazole for Prevention of Aspergillus Infections in Peripheral Blood Stem Cell and Marrow Transplant Recipients Completed NCT00003883 Phase 3 fluconazole;itraconazole
25 Allogeneic Blood or Marrow Transplantation for Hematologic Malignancy and Aplastic Anemia Completed NCT00003816 Phase 2, Phase 3 busulfan;carboplatin;cyclophosphamide;etoposide;fludarabine phosphate;melphalan;thiotepa
26 A Phase III Double-Blind Equivalence Study of Two Different Formulations of Slow-Release Morphine Followed by a Randomization Between Dextromethorphan or Placebo Plus Statex SR for Chronic Cancer Pain Relief in Terminally Ill Patients Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
27 A Randomized Double-Blind Placebo-Controlled Phase III Study To Evaluate The Safety And Efficacy Of Palivizumab Combined With Aerosolized Ribavirin Compared To Ribavirin Alone To Treat RSV Pneumonia In Patients With Bone Marrow Transplants (BMT) Completed NCT00014391 Phase 3 ribavirin
28 The Effects of Procrit (Epoetin Alfa) on Hemoglobin Symptom Distress and Quality of Life During Chemotherapy in Lymphoma Patients With Mild to Moderate Anemia A Multicenter Trial Completed NCT00003341 Phase 3
29 A Phase III Multicenter Study of Cytomegalovirus Prophylaxis With Valacyclovir for the Prevention of Serious Fungal and Bacterial Infections Among Cytomegalovirus Seronegative Recipients of Cytomegalovirus Seropositive Sx Stem Cell Transplants Completed NCT00045292 Phase 3 acyclovir;acyclovir sodium;valacyclovir
30 Music Video and Adolescent/Young Adult Resilience During Transplant Completed NCT00305851 Phase 3
31 Phase III Trial of T Lymphocyte Depletion by Elutriation and CD34 Add-Back for Allogeneic Bone Marrow Transplantation Completed NCT00265837 Phase 3
32 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
33 Vancomycin Versus Placebo in Persistently Febrile Granulocytopenic Patients Given Piperacillin/Tazobactam Completed NCT00003805 Phase 3 piperacillin sodium;piperacillin-tazobactam;tazobactam sodium;vancomycin
34 A Phase III, Randomized Study of the Effects of Parenteral Iron, Oral Iron, or No Iron Supplementation on the Erythropoietic Response to Darbepoetin Alfa for Cancer Patients With Chemotherapy-Associated Anemia Completed NCT00661999 Phase 3 sodium ferric gluconate complex in sucrose
35 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
36 A Strategic Study to Determine the Optimal Moment to Initiate Systemic Antifungal Therapy With Ambisome in Granulocytopenic Cancer Patients With Unexplained Fever Refractory to Empirical Antibacterials Completed NCT00003938 Phase 3 liposomal amphotericin B
37 A Multicenter, Double-Blind, Randomized, Comparative Study To Evaluate The Safety, Tolerability, And Efficacy Of MK-0991 Versus (Amphotericin B) Liposome For Injection As Empirical Therapy In Patients With Persistent Fever And Neutropenia Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
38 The Use of American Ginseng (Panax Quinquefolius) to Improve Cancer-Related Fatigue: A Randomized, Double-Blind, Placebo-Controlled Phase III Study Completed NCT00719563 Phase 3 American ginseng
39 Phase III Randomized Trial of an Opioid Titration Order Sheet Compared to Standard of Care in Patients With Cancer Related Pain. Completed NCT00666211 Phase 3
40 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
41 Randomized Phase III Trial Comparing Two Different Rituximab Dosing Regimens For Patients With Low Tumor Burden Indolent Non-Hodgkin's Lymphoma Active, not recruiting NCT00075946 Phase 3
42 Peripheral Blood Stem Cell Transplantation in Patients With Advanced Malignancies Eligible for Allogeneic Transplantation From Matched Related Donors: A Randomized Study of Cyclosporine/Methotrexate or Cyclosporine/T-Cell Depletion (CD34 Cell Selection) for GVHD Prophylaxis Terminated NCT00003056 Phase 3 cyclosporine;cyclosporine and methotrexate
43 A Randomized Study to Evaluate The Efficacy of Mycophenolate Mofetil Added to The Systemic Immunosuppressive Regimen First Used For Treatment of Chronic Graft-Versus-Host Disease Terminated NCT00089141 Phase 3 mycophenolate mofetil;placebo
44 Randomized, Double Blinded, Placebo-Controlled Trial of Antibacterial Prophylaxis for the Prevention of Bacterial Infections in the Post-Engraftment Phase After Allogeneic Hematopoeitic Stem Cell Transplantation Terminated NCT00324324 Phase 3 moxifloxacin hydrochloride;Placebo
45 Phase I/II Study to Assess the Safety and Efficacy of Low Doses of Beta LT in Patients With B-Cell Lymphoma Unknown status NCT00007839 Phase 1, Phase 2 beta alethine
46 Phase II Study of Arsenic Trioxide in Relapsed/Refractory Acute Leukemia and Blast Crisis of Chronic Myeloid Leukemia Unknown status NCT00003885 Phase 2 arsenic trioxide
47 Phase II Study of High-Dose Cytarabine, Cisplatin, and Dexamethasone Followed By Cyclophosphamide, Etoposide, Total Body Irradiation, and Autologous Bone Marrow Rescue in Patients With Relapsed or Refractory Non-Hodgkin's Lymphoma Unknown status NCT00002481 Phase 2 cisplatin;cyclophosphamide;cytarabine;dexamethasone;etoposide
48 Allograft of Hematopoietic Stem Cells With Reduced-intensity Conditioning From a HLA-haploidentical Family Donor: Phase II Study of Combined Immunosuppression Before and After Transplantation Unknown status NCT00740467 Phase 2 busulfan;cyclophosphamide;cyclosporine;fludarabine phosphate;mycophenolate mofetil
49 Safety and Efficacy of Sequential Treatment With a Combination of Rituximab, Fludarabine and Cyclophosphamide Followed by Zevalin (Rituximab and Y-Ibritumomab Tiuxetan) - A Phase I/II Study for Treatment of Patients With Relapsed Indolent and Transformed CD20-Positive B-Cell Non-Hodgkin's-Lymphoma Ineligible for High-Dose Chemo(Radio)Therapy Supported by Autologous Peripheral Blood Stem-Cells Unknown status NCT00397800 Phase 1, Phase 2 cyclophosphamide;fludarabine phosphate
50 Transplantation Using Umbilical Cord And Placental Blood Unknown status NCT00008164 Phase 2

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
Sources

Genetic Tests for Splenic Marginal Zone Lymphoma

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Sources

Anatomical Context for Splenic Marginal Zone Lymphoma

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The Foundational Model of Anatomy Ontology organs/tissues related to Splenic Marginal Zone Lymphoma:

19
Spleen Comprised Of B-Cells In Place Of White Pulp

MalaCards organs/tissues related to Splenic Marginal Zone Lymphoma:

40
B Cells, Bone, Bone Marrow, Spleen, T Cells, Lymph Node, Liver
Sources

Publications for Splenic Marginal Zone Lymphoma

About this section

Articles related to Splenic Marginal Zone Lymphoma:

(show top 50) (show all 533)
# Title Authors PMID Year
1
BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system. 61
32458259 2020
2
The Diagnostic Utility of Splenectomy in Idiopathic Splenomegaly. 61
32394013 2020
3
Atypical course of an infective endocarditis in a patient with complex congenital heart disease, chronic hepatitis B and splenic marginal zone lymphoma. 61
32426956 2020
4
Splenic Marginal Zone Lymphoma in Turkey: Association with Hepatitis B Instead of Hepatitis C Virus as an Etiologic and Possible Prognostic Factor - A Multicenter Cohort Study 61
31630512 2020
5
Molecular characterization of sorted malignant B cells from patients clinically identified with mantle cell lymphoma. 61
32173361 2020
6
Acquired C1-inhibitor deficiency due to splenic marginal zone lymhoma: Case Report. 61
32228367 2020
7
Cerebral HSV-1 Vasculitis as a Fatal Complication of Immunosuppression in Non-Hodgkin´s Lymphoma: A Case Report and Review of the Literature. 61
32151046 2020
8
Proposed rituximab biosimilar BCD-020 versus reference rituximab for treatment of patients with indolent non-Hodgkin lymphomas: An international multicenter randomized trial. 61
31724191 2020
9
Identification of a Splenic Marginal Zone Lymphoma Signature: Preliminary Findings With Diagnostic Potential. 61
32457837 2020
10
Rare Causes of Isolated and Progressive Splenic Lesions: Challenges in Differential Diagnosis, Evaluation, and Treatment of Primary Splenic Lymphomas. 61
32565679 2020
11
Successful Surgical Removal of the Largest Known Spleen. 61
32158587 2020
12
Splenectomy in Lymphoproliferative Disorders: A Single Eastern European Center Experience. 61
31892196 2019
13
Contemporary management of nodal and primary splenic marginal zone lymphoma. 61
31619091 2019
14
Deregulated NKL Homeobox Genes in B-Cell Lymphoma. 61
31779217 2019
15
Successful rechallenge of rituximab following severe rituximab-induced acute thrombocytopenia in a patient with splenic marginal zone lymphoma. 61
31766968 2019
16
A Case of Acquired Angioedema with Low C1 Inhibitor (C1-INH) Associated with Splenic Marginal Zone Lymphoma. 61
31588119 2019
17
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-2. Marginal zone lymphoma (MALT lymphoma/extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and splenic marginal zone lymphoma). 61
31428970 2019
18
A Case of Rituximab-Induced Acute Thrombocytopenia in a Patient with Splenic Marginal Zone Lymphoma and Chronic Hepatitis C Virus Infection. 61
31541071 2019
19
Splenic Marginal Zone Lymphoma with Prominent Myelofibrosis Mimicking Triple-Negative Primary Myelofibrosis. 61
31762757 2019
20
Practical Applications in Immunohistochemistry: An Immunophenotypic Approach to the Spleen. 61
30917045 2019
21
Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy: A case report and review of the literature. 61
31306619 2019
22
Epidermotropic CXCR3 positive marginal zone lymphoma: a distinctive clinical histopathological entity potentially originating in the skin; it does not always indicate splenic marginal zone lymphoma. 61
31450275 2019
23
Systematic Review of Somatic Mutations in Splenic Marginal Zone Lymphoma. 61
31320741 2019
24
Transformation of a splenic marginal zone lymphoma into classic Hodgkin lymphoma. 61
30814198 2019
25
Splenic Marginal Zone B-Cell Lymphoma With Splenic Infarction in a Patient With Cold Agglutinin Disease. 61
30888998 2019
26
Central nervous system involvement in primary bone marrow or splenic marginal zone lymphoma: Report of two cases and review of the literature. 61
30592785 2019
27
Lymphomagenic properties of a HIV p17 variant derived from a splenic marginal zone lymphoma occurred in a HIV-infected patient. 61
30261551 2019
28
Bone marrow-derived mesenchymal stem/stromal cells from patients with splenic marginal zone lymphoma are intrinsically impaired and influence the malignant B-cells. 61
29966468 2019
29
Splenic marginal zone lymphoma treated with laparoscopic splenectomy: A case report. 61
31759300 2019
30
Splenic marginal zone lymphoma leads to sinistral portal hypertension. 61
30549195 2019
31
Stereotyped B Cell Receptor Immunoglobulins in B Cell Lymphomas. 61
30779034 2019
32
Clonal villous lymphocytes and plasma cells in splenic marginal zone lymphoma with plasmacytic differentiation. 61
30588460 2018
33
Efficacy of bendamustine and rituximab in splenic marginal zone lymphoma: results from the phase II BRISMA/IELSG36 study. 61
30407629 2018
34
18F-FDG PET/CT in splenic marginal zone lymphoma. 61
29500652 2018
35
Splenic marginal zone lymphoma with a de novo t(8;14)(q24;q32) and a prolymphocytoid evolution responsive to rituximab-bendamustine. 61
29728736 2018
36
Hyperviscosity syndrome in splenic marginal zone lymphoma. 61
30309878 2018
37
Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. 61
30073422 2018
38
Remission of Cutaneous Involvement in Splenic Marginal Zone Lymphoma after Splenectomy. 61
29856467 2018
39
Rituximab monotherapy in splenic marginal zone lymphoma: prolonged responses and potential benefit from maintenance. 61
29914978 2018
40
Deciphering splenic marginal zone lymphoma pathogenesis: the proposed role of microRNA. 61
30042829 2018
41
Splenic marginal zone lymphoma relapsing as miliary lung mottling: an unusual presentation. 61
29963513 2018
42
A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation. 61
29075958 2018
43
Surgical management of splenic marginal zone lymphoma. 61
29043543 2018
44
Toll-like receptor expression and function differ between splenic marginal zone B cell lymphoma and splenic diffuse red pulp B cell lymphoma. 61
29805758 2018
45
Should rituximab replace splenectomy in the management of splenic marginal zone lymphoma? 61
29452668 2018
46
Idelalisib and Rituximab in 17p Deletion-Positive Splenic Marginal Zone Lymphoma. 61
29523661 2018
47
Intravenous immunoglobulin-induced acute thrombocytopenia. 61
29193103 2018
48
Spectrum and immunophenotyping of 653 patients with B-cell chronic lymphoproliferative disorders in China: A single-centre analysis. 61
28752619 2018
49
Hepatitis C virus infection is an independent prognostic factor in follicular lymphoma. 61
29416725 2018
50
Recurrent autoimmune hemolytic anemia in splenic marginal zone lymphoma. 61
30181837 2018
Sources

Variations for Splenic Marginal Zone Lymphoma

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Cosmic variations for Splenic Marginal Zone Lymphoma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM105231581 MYD88 haematopoietic and lymphoid tissue,spleen,lymphoid neoplasm,marginal zone lymphoma c.818T>C p.L273P 3:38141150-38141150 0

Copy number variations for Splenic Marginal Zone Lymphoma from CNVD:

7 (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 MUC3 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 MLKN1 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

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

Pathways for Splenic Marginal Zone Lymphoma

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

(show all 27)
# Super pathways Score Top Affiliating Genes
1
TNFR1 Pathway 63
Show member pathways
 12.96 TRAF3 NFKB2 GNG11 CREBBP BTK BIRC3
2
Toll-like Receptor Signaling Pathway 36 1
Show member pathways
 12.94 TRAF3 SYK NFKB2 CREBBP CDK6 CD40
3
PI3K-Akt signaling pathway 36
Show member pathways
 12.87 TRAF3 SYK NOTCH2 GNG11 CREBBP CDK6
4
Human cytomegalovirus infection 36
Show member pathways
 12.82 TRAF3 SYK GNG11 CREBBP CDK6
5
Pathways in cancer 36
12.64 TRAF3 NOTCH2 NFKB2 GNG11 CREBBP CDK6
6
NF-KappaB Family Pathway 63
Show member pathways
 12.59 TRAF3 NFKB2 MALT1 CREBBP CD40
7
Measles 36
Show member pathways
 12.57 TRAF3 SYK NFKB2 CREBBP CDK6 CD40
8
Immune response NFAT in immune response 22
Show member pathways
 12.53 SYK NFKB2 MALT1 GNG11 CD40 BTK
9
Notch Signaling Pathway (WikiPathways) 1
Show member pathways
 12.44 NOTCH2 NFKB2 CREBBP BCL6
10
MicroRNAs in cancer 36
12.43 NOTCH2 MIR26B FOXP1 CREBBP CDK6
11
RANK Signaling in Osteoclasts 63
Show member pathways
 12.32 TRAF3 SYK NFKB2 BIRC3
12
CLEC7A (Dectin-1) signaling 65
Show member pathways
 12.31 SYK NFKB2 MALT1 CREBBP
13
B cell receptor signaling pathway (KEGG) 36
Show member pathways
 12.29 SYK MALT1 BTK BCL6
14
Viral carcinogenesis 36
12.18 TRAF3 SYK NFKB2 CREBBP CDK6
15
B Cell Receptor Signaling Pathway (sino) 66
Show member pathways
 12.16 SYK NFKB2 MALT1 CD40 BTK BCL6
16
Transcriptional misregulation in cancer 36
12.15 PAX5 CD40 BIRC3 BCL6
17
NF-kappaB Signaling 4
12.07 TRAF3 SYK PAX5 NFKB2 MALT1 BTK
18
C-type lectin receptor signaling pathway 36
Show member pathways
 12.04 SYK NFKB2 MALT1 CREBBP BTK
19
Hematopoietic Stem Cells and Lineage-specific Markers 64
11.87 SDC1 NOTCH2 CD5
20
Small cell lung cancer 36
11.74 TRAF3 CDK6 BIRC3
21
LT-BetaR Pathway 63
Show member pathways
 11.67 TRAF3 NFKB2 CREBBP
22
C-MYB transcription factor network 1
11.65 PAX5 CREBBP CDK6 BIRC3
23
Apoptosis and survival Anti-apoptotic TNFs/NF-kB/Bcl-2 pathway 22
Show member pathways
 11.6 TRAF3 NFKB2 CD40 BIRC3
24
NF-kB(NFkB) Pathway 66
11.56 TRAF3 SYK NFKB2 MALT1 BTK
25
Primary immunodeficiency 36
11.3 CD40 BTK AICDA
26
CD40/CD40L signaling 1
11.18 TRAF3 CD40 BIRC3
27
NF-kappa B signaling pathway 36
11.18 TRAF3 SYK NFKB2 MALT1 CD40 BTK
Sources

GO Terms for Splenic Marginal Zone Lymphoma

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

# Name GO ID Score Top Affiliating Genes
1 CD40 receptor complex GO:0035631 8.62 TRAF3 CD40

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

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.93 TRAF3 SYK NFKB2 MALT1 BTK
2 negative regulation of transcription by RNA polymerase II GO:0000122 9.8 PAX5 NOTCH2 NFKB2 KLF2 CREBBP CDK6
3 Notch signaling pathway GO:0007219 9.73 NOTCH2 CREBBP CDK6
4 stimulatory C-type lectin receptor signaling pathway GO:0002223 9.72 SYK MALT1 CREBBP
5 regulation of inflammatory response GO:0050727 9.7 FOXP1 BIRC3 BCL6
6 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.67 NFKB2 MALT1 CD40 BTK
7 I-kappaB kinase/NF-kappaB signaling GO:0007249 9.63 NFKB2 BTK BIRC3
8 positive regulation of type I interferon production GO:0032481 9.58 SYK NFKB2 CREBBP
9 positive regulation of B cell differentiation GO:0045579 9.51 SYK BTK
10 lateral ventricle development GO:0021670 9.49 PAX5 CDK6
11 negative regulation of B cell apoptotic process GO:0002903 9.48 FOXP1 BCL6
12 germinal center formation GO:0002467 9.43 NFKB2 BCL6
13 B cell activation GO:0042113 9.33 MALT1 CD40 BTK
14 cellular response to molecule of fungal origin GO:0071226 9.32 SYK BTK
15 TRIF-dependent toll-like receptor signaling pathway GO:0035666 9.13 TRAF3 CD40 BIRC3
16 regulation of apoptotic process GO:0042981 9.02 TRAF3 MALT1 CREBBP BIRC3 BCL6

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.58 TRAF3 SYK SDC1 PAX5 NOTCH2 NFKB2
Sources

Sources for Splenic Marginal Zone Lymphoma

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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