WM
MCID: LYM012
MIFTS: 62

Lymphoplasmacytic Lymphoma (WM)

Categories: Blood diseases, Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Lymphoplasmacytic Lymphoma

MalaCards integrated aliases for Lymphoplasmacytic Lymphoma:

Name: Lymphoplasmacytic Lymphoma 12 53 37 15 38
Waldenstrom Macroglobulinemia 12 53 25 59 29 55 44 40 17 72
Macroglobulinemia of Waldenstrom 53 25
Malignant Lymphoma - Lymphoplasmacytic 72
Waldenstrom's Macroglobulinaemia 53
Waldenström's Macroglobulinemia 75
Waldenstrom's Macroglobulinemia 25
Waldenström Macroglobulinemia 25
Waldenstrom's Syndrome 53
Wm 25

Characteristics:

Orphanet epidemiological data:

59
waldenstrom macroglobulinemia
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe),1-9/100000 (France),1-9/1000000 (United States),1-9/1000000 (United Kingdom),1-9/1000000 (Spain),1-9/100000 (Europe); Age of onset: Elderly; Age of death: elderly;

Classifications:



External Ids:

Disease Ontology 12 DOID:0050747
KEGG 37 H00011
ICD9CM 35 273.3
MeSH 44 D008258
SNOMED-CT 68 35562000
ICD10 33 C88.0
MESH via Orphanet 45 D008258
ICD10 via Orphanet 34 C88.0
UMLS via Orphanet 73 C0024419
Orphanet 59 ORPHA33226
UMLS 72 C0024419 C0334633 C2936755

Summaries for Lymphoplasmacytic Lymphoma

Genetics Home Reference : 25 Waldenström macroglobulinemia is a rare blood cell cancer characterized by an excess of abnormal white blood cells called lymphoplasmacytic cells in the bone marrow. This condition is classified as a lymphoplasmacytic lymphoma. The abnormal cells have characteristics of both white blood cells (lymphocytes) called B cells and of more mature cells derived from B cells known as plasma cells. These abnormal cells produce excess amounts of IgM, a type of protein known as an immunoglobulin; the overproduction of this large protein is how the condition got its name ("macroglobulinemia"). Waldenström macroglobulinemia usually begins in a person's sixties and is a slow-growing (indolent) cancer. Some affected individuals have elevated levels of IgM and lymphoplasmacytic cells but no symptoms of the condition; in these cases, the disease is usually found incidentally by a blood test taken for another reason. These individuals are diagnosed with smoldering (or asymptomatic) Waldenström macroglobulinemia. It can be several years before this form of the condition progresses to the symptomatic form. Individuals with symptomatic Waldenström macroglobulinemia can experience general symptoms such as fever, night sweats, and weight loss. Several other signs and symptoms of the condition are related to the excess IgM, which can thicken blood and impair circulation, causing a condition known as hyperviscosity syndrome. Features related to hyperviscosity syndrome include bleeding in the nose or mouth, blurring or loss of vision, headache, dizziness, and difficulty coordinating movements (ataxia). In some affected individuals, the IgM proteins clump together in the hands and feet, where the body temperature is cooler than at the center of the body. These proteins are then referred to as cryoglobulins, and their clumping causes a condition known as cryoglobulinemia. Cryoglobulinemia can lead to pain in the hands and feet or episodes of Raynaud phenomenon, in which the fingers and toes turn white or blue in response to cold temperatures. The IgM protein can also build up in organs such as the heart and kidneys, causing a condition called amyloidosis, which can lead to heart and kidney problems. Some people with Waldenström macroglobulinemia develop a loss of sensation and weakness in the limbs (peripheral neuropathy). Doctors are unsure why this feature occurs, although they speculate that the IgM protein attaches to the protective covering of nerve cells (myelin) and breaks it down. The damaged nerves cannot carry signals normally, leading to neuropathy. Other features of Waldenström macroglobulinemia are due to the accumulation of lymphoplasmacytic cells in different tissues. For example, accumulation of these cells can lead to an enlarged liver (hepatomegaly), spleen (splenomegaly), or lymph nodes (lymphadenopathy). In the bone marrow, the lymphoplasmacytic cells interfere with normal blood cell development, causing a shortage of normal blood cells (pancytopenia). Excessive tiredness (fatigue) due to a reduction in red blood cells (anemia) is common in affected individuals. People with Waldenström macroglobulinemia have an increased risk of developing other cancers of the blood or other tissues.

MalaCards based summary : Lymphoplasmacytic Lymphoma, also known as waldenstrom macroglobulinemia, is related to waldenstroem's macroglobulinemia and macroglobulinemia. An important gene associated with Lymphoplasmacytic Lymphoma is MYD88 (MYD88 Innate Immune Signal Transduction Adaptor), and among its related pathways/superpathways are Transcriptional misregulation in cancer and Akt Signaling. The drugs Lenograstim and Thiotepa have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and bone marrow, and related phenotypes are leukemia and lymphoma

Disease Ontology : 12 A B-cell lymphocytic neoplasm characterized by an uncontrolled increase of B-cells.

NIH Rare Diseases : 53 Waldenstrom macroglobulinemia is a chronic, slow-growing lymphoproliferative disorder. It usually affects older adults and is primarily found in the bone marrow, although lymph nodes and the spleen may be involved. Affected individuals have a high level of an antibody called immunoglobulin M (IgM) in their blood, which can cause thickening of the blood (hyperviscosity). Although some individuals initially do not have symptoms and are diagnosed from routine blood work, common symptoms may include weakness, appetite loss and weight loss. Other symptoms may include peripheral neuropathy, fever, Raynaud's phenomenon, and mental status changes. Hyperviscosity of the blood may cause nosebleeds, headaches, dizziness, and blurring or loss of vision. The cause of the condition is not known but environmental, genetic, and viral factors have been suggested. There have been some reports of familial cases suggesting a genetic predisposition. Treatment is often reserved for those with symptoms and may include various medications including corticosteroids, alkylating agents, biologic response modifiers and purine analogues.

KEGG : 37
Lymphoplasmacytic lymphoma (LPL)/Waldenstrom's macroglobulinemia (WM) is a non-Hodgkin lymphoma (NHL) subtype characterized by small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow, lymph nodes, and the spleen. WM can be distinguished clinically from LPL on the basis of a detectable monoclonal (IgM) immunoglobulin spike in serum. In the general population, LPL/WM is a very rare disease, accounting for only 1%-2% of all hematologic malignancies, reflected in an incidence rate of 3-4 cases per million people per year. The t(9;14)(p13;q32) is present in near 50% of cases of LPL. This chromosomal translocation involves a junction between 9p13 and the switch micro region of the Ig heavy chain locus on 14q32. The 9p13 breakpoint contains the PAX-5 gene which encodes a B-cell specific transcription factor involved in the control of B-cell proliferation and differentiation. The translocation causes the juxtaposition of the PAX-5 gene to the IgH locus in the opposite direction of transcription, resulting in an 11-fold over-expression of PAX-5 mRNA.

Wikipedia : 75 Waldenstrom's macroglobulinemia (WM), is a type of cancer affecting two types of B cells:... more...

Related Diseases for Lymphoplasmacytic Lymphoma

Diseases related to Lymphoplasmacytic Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 491)
# Related Disease Score Top Affiliating Genes
1 waldenstroem's macroglobulinemia 33.9 MYD88 MAG CXCR4
2 macroglobulinemia 31.5 PAX5 MYD88 CXCR4 CXCL12 CD40LG
3 diffuse large b-cell lymphoma 31.0 MYD88 MIR155 IRF4
4 heavy chain disease 31.0 PAX5 MYD88
5 lymphoma, hodgkin, classic 30.7 PTPRC PAX5 MIR155
6 marginal zone b-cell lymphoma 30.6 MYD88 IRF4 CD40LG CCND1
7 monoclonal paraproteinemia 30.6 MYD88 CCND1
8 follicular lymphoma 30.3 MIR155 IRF4 CCND1
9 leukemia, acute myeloid 30.1 PTPRC MIR184 MIR155 CXCR4 CXCL12
10 cll/sll 30.1 ZAP70 PAX5 CCND1
11 plasma cell leukemia 30.1 IRF4 CCND1
12 agammaglobulinemia, x-linked 29.8 CXCL12 CD40LG CD40
13 toxoplasmosis 29.8 MYD88 CD40LG CD40
14 lymphoma, mucosa-associated lymphoid type 29.4 IRF4 CD40 CCND1
15 mature b-cell neoplasm 29.1 PTPRC MAG CD40
16 burkitt lymphoma 29.0 PAX5 MS4A1 MIR155 CD40LG CD40
17 mantle cell lymphoma 28.7 PTPRC PAX5 CD40 CCND1
18 myeloma, multiple 28.5 PTPRC IRF4 CXCR4 CXCL12 CD40 CCND1
19 leukemia, chronic lymphocytic 27.7 ZAP70 PAX5 MS4A1 CXCR4 CXCL12 CD40LG
20 lymphoma, non-hodgkin, familial 27.4 PTPRC PAX5 MS4A1 IRF4 CXCR4 CXCL12
21 lymphoplasmacytic lymphoma without igm production 12.4
22 weill-marchesani syndrome 12.0
23 macroglobulinemia, waldenstrom 1 11.9
24 tibia, hypoplasia or aplasia of, with polydactyly 11.5
25 williams-beuren syndrome 11.5
26 microspherophakia and/or megalocornea, with ectopia lentis and with or without secondary glaucoma 11.2
27 weill-marchesani syndrome 2 11.2
28 weill-marchesani syndrome 3 11.2
29 lymphoma 10.9
30 b-cell lymphoma 10.6
31 leukemia 10.5
32 aids dementia complex 10.5 CXCR4 CXCL12
33 primary cutaneous diffuse large b-cell lymphoma, leg type 10.4 MYD88 IRF4
34 immunodysregulation, polyendocrinopathy, and enteropathy, x-linked 10.4 CXCR4 CXCL12 CD40LG
35 lymphocytic leukemia 10.4
36 temporal lobe epilepsy 10.4
37 traumatic brain injury 10.4
38 plasma protein metabolism disease 10.4 MYD88 MAG CXCR4
39 myeloid leukemia 10.4
40 leukemia, b-cell, chronic 10.4
41 intraocular lymphoma 10.4 PAX5 CXCR4
42 sarcoma 10.3
43 spindle cell sarcoma 10.3
44 hepatocellular carcinoma 10.3
45 acute leukemia 10.3
46 testicular lymphoma 10.3 PTPRC MYD88
47 splenomegaly 10.3
48 primary central nervous system lymphoma 10.3 PAX5 MYD88
49 retinal detachment 10.2
50 ocular motor apraxia 10.2

Graphical network of the top 20 diseases related to Lymphoplasmacytic Lymphoma:



Diseases related to Lymphoplasmacytic Lymphoma

Symptoms & Phenotypes for Lymphoplasmacytic Lymphoma

Human phenotypes related to Lymphoplasmacytic Lymphoma:

59 32 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 leukemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001909
2 lymphoma 59 32 hallmark (90%) Very frequent (99-80%) HP:0002665
3 monoclonal immunoglobulin m proteinemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0005508
4 respiratory insufficiency 59 32 frequent (33%) Frequent (79-30%) HP:0002093
5 hypercoagulability 59 32 frequent (33%) Frequent (79-30%) HP:0100724
6 pallor 59 32 frequent (33%) Frequent (79-30%) HP:0000980
7 gingival bleeding 59 32 frequent (33%) Frequent (79-30%) HP:0000225
8 vertigo 59 32 frequent (33%) Frequent (79-30%) HP:0002321
9 abnormality of neutrophils 59 32 frequent (33%) Frequent (79-30%) HP:0001874
10 normocytic anemia 59 32 frequent (33%) Frequent (79-30%) HP:0001897
11 ataxia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001251
12 hearing impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0000365
13 splenomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0001744
14 hepatomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0002240
15 malabsorption 59 32 occasional (7.5%) Occasional (29-5%) HP:0002024
16 reduced consciousness/confusion 59 32 occasional (7.5%) Occasional (29-5%) HP:0004372
17 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
18 fever 59 32 occasional (7.5%) Occasional (29-5%) HP:0001945
19 cranial nerve paralysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0006824
20 fatigue 59 32 occasional (7.5%) Occasional (29-5%) HP:0012378
21 peripheral neuropathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0009830
22 congestive heart failure 59 32 occasional (7.5%) Occasional (29-5%) HP:0001635
23 urticaria 59 32 occasional (7.5%) Occasional (29-5%) HP:0001025
24 pulmonary infiltrates 59 32 occasional (7.5%) Occasional (29-5%) HP:0002113
25 gastrointestinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0002239
26 memory impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0002354
27 anorexia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002039
28 migraine 59 32 occasional (7.5%) Occasional (29-5%) HP:0002076
29 purpura 59 32 occasional (7.5%) Occasional (29-5%) HP:0000979
30 recurrent infections 59 32 occasional (7.5%) Occasional (29-5%) HP:0002719
31 proptosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000520
32 diarrhea 59 32 occasional (7.5%) Occasional (29-5%) HP:0002014
33 epistaxis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000421
34 vasculitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002633
35 stroke 59 32 occasional (7.5%) Occasional (29-5%) HP:0001297
36 periorbital edema 59 32 occasional (7.5%) Occasional (29-5%) HP:0100539
37 lymphadenopathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0002716
38 cutis marmorata 59 32 occasional (7.5%) Occasional (29-5%) HP:0000965
39 retinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0000573
40 pleural effusion 59 32 occasional (7.5%) Occasional (29-5%) HP:0002202
41 elevated erythrocyte sedimentation rate 59 32 occasional (7.5%) Occasional (29-5%) HP:0003565
42 cryoglobulinemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0100778
43 multifocal epileptiform discharges 59 32 occasional (7.5%) Occasional (29-5%) HP:0010841
44 pedal edema 32 occasional (7.5%) HP:0010741
45 abnormal retinal vascular morphology 32 occasional (7.5%) HP:0008046
46 abnormality of the retinal vasculature 59 Occasional (29-5%)
47 edema of the lower limbs 59 Occasional (29-5%)

MGI Mouse Phenotypes related to Lymphoplasmacytic Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.93 CCND1 CD40 CD40LG CXCL12 CXCR4 IRF4
2 immune system MP:0005387 9.73 CCND1 CD40 CD40LG CXCL12 CXCR4 IRF4
3 neoplasm MP:0002006 9.1 CCND1 CXCR4 IRF4 MYD88 PAX5 PTPRC

Drugs & Therapeutics for Lymphoplasmacytic Lymphoma

Drugs for Lymphoplasmacytic Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 325)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenograstim Approved, Investigational Phase 4 135968-09-1
2
Thiotepa Approved, Investigational Phase 4 52-24-4 5453
3 Orange Approved Phase 3
4
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
5
Chlorambucil Approved Phase 3 305-03-3 2708
6
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
7
Ondansetron Approved Phase 3 99614-02-5 4595
8
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
9
Morphine Approved, Investigational Phase 3 57-27-2 5288826
10
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
11
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
12
Darbepoetin alfa Approved, Investigational Phase 3 11096-26-7, 209810-58-2
13
Caspofungin Approved Phase 3 179463-17-3, 162808-62-0 468682 2826718
14
Tazobactam Approved Phase 3 89786-04-9 123630
15
Piperacillin Approved Phase 3 66258-76-2 43672
16
Vancomycin Approved Phase 3 1404-90-6 441141 14969
17
Amphotericin B Approved, Investigational Phase 3 1397-89-3 14956 5280965
18
Etoposide Approved Phase 2, Phase 3 33419-42-0 36462
19
Carboplatin Approved Phase 2, Phase 3 41575-94-4 10339178 498142 38904
20
Idelalisib Approved Phase 3 870281-82-6
21
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
22
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
23 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
24 Anesthetics, Dissociative Phase 3
25 Gastrointestinal Agents Phase 3
26 Antiemetics Phase 3
27 Autonomic Agents Phase 3
28 Peripheral Nervous System Agents Phase 3
29 Antifungal Agents Phase 3
30 Neurotransmitter Agents Phase 3
31 Hematinics Phase 3
32 Tranquilizing Agents Phase 3
33 Central Nervous System Depressants Phase 3
34 Psychotropic Drugs Phase 3
35 Adjuvants, Immunologic Phase 3
36 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
37 Analgesics Phase 3
38 Cola Phase 3
39 Liver Extracts Phase 3
40 Antiparasitic Agents Phase 3
41 Antiprotozoal Agents Phase 3
42 Anti-Anxiety Agents Phase 3
43 Serotonin Antagonists Phase 3
44 Analgesics, Opioid Phase 3
45 Serotonin Agents Phase 3
46 Antipruritics Phase 3
47 Antipsychotic Agents Phase 3
48 Narcotics Phase 3
49 Respiratory System Agents Phase 3
50 Antitussive Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 487)
# 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 The Efficacy of Thalidomide Plus Cyclophosphamide and Dexamethasone Following by Thalidomide and Prednisone Maintenance Therapy for the Newly Diagnosed Waldenström Macroglobulinemia - a Prospective Multicentre Phase Ⅳ Trial From China Recruiting NCT02844309 Phase 4 Thalidomide
3 The Efficacy of Cyclophosphamide Plus Dexamethasone Combinated With Rituximab or Bortezomib for the Newly Diagnosed Waldenström Macroglobulinemia - a Prospective Multicentre Randomized Control Trial From China Recruiting NCT02844322 Phase 4 Bortezomib;Rituximab
4 Autologous Stem-cell Transplantation Versus Conventional Chemotherapy for High Risk Waldenström Macroglobulinemia - a Prospective Multicentre Phase Ⅳ Trial From China Recruiting NCT02844361 Phase 4
5 A Single Arm, Multicenter, Phase 4 Study of the Bruton's Tyrosine Kinase (BTK) Inhibitor, Ibrutinib (PCI-32765) in Chinese Subjects With Relapse or Refractory Waldenström's Macroglobulinemia Not yet recruiting NCT04042376 Phase 4 Ibrutinib
6 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
7 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
8 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
9 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
10 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
11 First-line R-CVP vs R-CHOP Induction Immunochemotherapy for Indolent Lymphoma and R Maintenance.A Multicentre, Phase III Randomized Study by the PLRG. Completed NCT00801281 Phase 3 Rituximab;Cyclophosphamide;Doxorubicin;Vincristine;Prednisone 1;Prednisone 2
12 A Phase III Study of Involved Field Radiation Therapy (IFRT) in Patients With Histologically Aggressive Non-Hodgkin's Lymphoma Following High Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation (ASCT) Completed NCT00031668 Phase 3
13 A Multi-center Phase III Study Comparing Nonmyeloablative Conditioning With TBI Versus Fludarabine/TBI for HLA-matched Related Hematopoietic Cell Transplantation for Treatment of Hematologic Malignancies Completed NCT00075478 Phase 3 Fludarabine Phosphate;Mycophenolate Mofetil;Cyclosporine
14 A Randomized Phase III Study to Determine the Most Promising Postgrafting Immunosuppression for Prevention of Acute GVHD After Unrelated Donor Hematopoietic Cell Transplantation Using Nonmyeloablative Conditioning for Patients With Hematologic Malignancies: A Multi-center Trial Completed NCT01231412 Phase 3 Cyclosporine;Fludarabine Phosphate;Mycophenolate Mofetil;Sirolimus
15 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
16 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
17 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
18 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
19 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
20 A Randomized, Open-Label, Multicenter Study Of Darbepoetin Alfa Administered Once Every Two Weeks (Q2W) Compared With rHuEPO Administered Once Every Week (QW) For The Treatment Of Anemia In Subjects With Non-Myeloid Malignancies Receiving Multiple Chemotherapy Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa
21 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
22 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
23 Vancomycin Versus Placebo in Persistently Febrile Granulocytopenic Patients Given Piperacillin/Tazobactam Completed NCT00003805 Phase 3 piperacillin sodium;piperacillin-tazobactam;tazobactam sodium;vancomycin
24 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
25 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
26 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
27 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
28 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
29 Efficacy and Safety of Ibrutinib in Patients With Chronic Lymphocytic Leukemia and Other Indolent B-cell Lymphomas Who Are Chronic Hepatitis B Virus Carriers or Occult Hepatitis B Virus Carriers Recruiting NCT02991638 Phase 3 Ibrutinib
30 Efficacy of First Line Dexamethasone, Rituximab and Cyclophosphamide (DRC) +/- Bortezomib for Patients With Waldenström's Macroglobulinemia Active, not recruiting NCT01788020 Phase 3 Dexamethasone, Rituximab, Cyclophosphamide;Dexamethasone, Rituximab, Cyclophosphamide, Bortezomib
31 iNNOVATE Study: A Randomized, Double-Blind, Placebo- Controlled, Phase 3 Study of Ibrutinib or Placebo in Combination With Rituximab in Subjects With Waldenström's Macroglobulinemia Active, not recruiting NCT02165397 Phase 3 Ibrutinib;Placebo;Rituximab
32 A Phase 3, Randomized, Open-Label, Multicenter Study Comparing the Efficacy and Safety of the Bruton's Tyrosine Kinase (BTK) Inhibitors BGB-3111 and Ibrutinib in Subjects With Waldenström's Macroglobulinemia (WM) Active, not recruiting NCT03053440 Phase 3 BGB-3111;Ibrutinib
33 Allogeneic Blood or Marrow Transplantation for Hematologic Malignancy and Aplastic Anemia Active, not recruiting NCT00003816 Phase 2, Phase 3 busulfan;carboplatin;cyclophosphamide;etoposide;fludarabine phosphate;melphalan;thiotepa
34 Randomised Phase II/III Study of Rituximab and Ibrutinib (RI) Versus Dexamethasone, Rituximab and Cyclophosphamide (DRC) as Initial Therapy for Waldenström's Macroglobulinaemia Not yet recruiting NCT04061512 Phase 2, Phase 3 Dexamethasone, cyclophosphamide, rituximab;Rituximab, ibrutinib
35 A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study Evaluating the Efficacy and Safety of Idelalisib (GS-1101) in Combination With Bendamustine and Rituximab for Previously Treated Indolent Non-Hodgkin Lymphomas Terminated NCT01732926 Phase 3 Idelalisib;Rituximab;Bendamustine;Placebo
36 A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study Evaluating the Efficacy and Safety of Idelalisib (GS-1101) in Combination With Rituximab for Previously Treated Indolent Non-Hodgkin Lymphomas Terminated NCT01732913 Phase 3 Placebo;Rituximab;Idelalisib
37 Phase I/II Study to Assess the Safety and Efficacy of Low Doses of Beta LT in Patients With Waldenstrom's Macroglobulinemia Unknown status NCT00041379 Phase 1, Phase 2 beta alethine
38 Study of Safety, Efficacy and Pharmacokinetics of CT-1530 in Patients With Relapsed or Refractory B Cell Non-Hodgkin Lymphoma, Chronic Lymphocytic Leukemia, and Waldenstrom's Macroglobulinemia Unknown status NCT02981745 Phase 1, Phase 2 CT-1530
39 A Phase II Study of Carfilzomib in Relapsed Waldenström's Macroglobulinemia (WM) IST-CAR-531 Unknown status NCT01813227 Phase 2 Carfilzomib;Rituximab;Dexamethasone
40 Velcade/Dexamethasone/Cyclophosphamide(PCD) Versus Rituximab /Dexamethasone/Cyclophosphamide(RCD) for the Treatment of Patients With Waldenstrom's Macroglobulinemia Unknown status NCT02971982 Phase 1, Phase 2 rituximab /Dexamethasone/cyclophosphamide;Velcade/Dexamethasone/cyclophosphamide
41 Phase II Study of Combination Bortezomib, Dexamethasone, and Rituximab in Previously Untreated Patients With Waldenstrom's Macroglobulinemia: A Multicenter Trial of the European Myeloma Network Unknown status NCT01046006 Phase 2 Bortezomib, Rituximab, Dexamethasone
42 A Single Arm, Phase II Study of the Anti-Blys Monoclonal Antibody, Belimumab in Symptomatic Waldenstroms Macroglobulinaemia Unknown status NCT01142011 Phase 2 Belimumab
43 Epigenetic Therapy With Valproic Acid, an HDAC Inhibitor, in Refractory/Relapsed Non-Hodgkin Lymphoma, Hodgkin's Disease and CLL Unknown status NCT01016990 Phase 2 valproic acid
44 A Phase I-II Trial of Brentuximab Vedotin Plus Rituximab as Frontline Therapy for Patients With CD30+ and/or EBV+ Lymphomas Unknown status NCT01805037 Phase 1, Phase 2 brentuximab vedotin
45 Myeloblative Therapy With Autologous Hematopoietic Stem Cell Transplantation in Patients With Multiple Myeloma and B-Cell Malignancies Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
46 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
47 A Phase II Study of Perifosine in Patients With Relapsed/Refractory Waldenstrom's Macroglobulinemia Completed NCT00422656 Phase 2 Perifosine
48 Phase 1/2 Open-Label, Multiple-dose, Dose-escalation Study to Evaluate the Safety and Tolerability of IMO-8400 in Patients With Relapsed or Refractory Waldenstrom's Macroglobulinemia Completed NCT02092909 Phase 1, Phase 2 IMO-8400
49 A Phase IIA Trial Testing the Efficacy of Bortezomib (Velcade(R)) in Patients With Advanced Waldenström Macroglobulinemia Completed NCT00777738 Phase 2 BORTEZOMIB
50 An Extension Study to Evaluate the Long-Term Safety, Tolerability, and Clinical Activity of IMO-8400 in Patients With Relapsed or Refractory Waldenström's Macroglobulinemia Who Completed Study 8400-401 Completed NCT02363439 Phase 1, Phase 2 IMO-8400 0.6 mg/kg/wk or 1.2 mg/kg twice weekly

Search NIH Clinical Center for Lymphoplasmacytic Lymphoma

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Bendamustine
Carfilzomib
Chlorambucil
Chlorambucil
Cyclophosphamide
Cyclophosphamide
Doxorubicin
Ibrutinib
Ibrutinib
Thalidomide
Thalidomide

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Lymphoplasmacytic Lymphoma cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: waldenstrom macroglobulinemia

Genetic Tests for Lymphoplasmacytic Lymphoma

Genetic tests related to Lymphoplasmacytic Lymphoma:

# Genetic test Affiliating Genes
1 Waldenstrom Macroglobulinemia 29 MYD88

Anatomical Context for Lymphoplasmacytic Lymphoma

MalaCards organs/tissues related to Lymphoplasmacytic Lymphoma:

41
B Cells, Bone, Bone Marrow, T Cells, Lymph Node, Spleen, Heart

Publications for Lymphoplasmacytic Lymphoma

Articles related to Lymphoplasmacytic Lymphoma:

(show top 50) (show all 840)
# Title Authors PMID Year
1
microRNA expression in the biology, prognosis, and therapy of Waldenström macroglobulinemia. 88
19074725 2009
2
Inherited and acquired variations in the hyaluronan synthase 1 (HAS1) gene may contribute to disease progression in multiple myeloma and Waldenstrom macroglobulinemia. 9 38
18815290 2008
3
The HMG-CoA inhibitor, simvastatin, triggers in vitro anti-tumour effect and decreases IgM secretion in Waldenstrom macroglobulinaemia. 9 38
18537966 2008
4
Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. 9 38
18452095 2008
5
Genomewide linkage screen for Waldenstrom macroglobulinemia susceptibility loci in high-risk families. 9 38
16960805 2006
6
Expression of inhibitor of apoptosis proteins in B-cell non-Hodgkin and Hodgkin lymphomas. 9 38
16983704 2006
7
Translocation t(9;14)(p13;q32) in cases of splenic marginal zone lymphoma. 9 38
16956840 2006
8
Distribution and ZAP-70 expression of WHO lymphoma categories in Shanxi, China: a review of 447 cases using a tissue microarray technique. 9 38
16280661 2005
9
Pentostatin/cyclophosphamide with or without rituximab: an effective regimen for patients with Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma. 9 38
16231851 2005
10
How we treat Waldenström's macroglobulinemia. 9 38
15642678 2005
11
Initial immunoglobulin M 'flare' after rituximab therapy in patients diagnosed with Waldenstrom macroglobulinemia: an Eastern Cooperative Oncology Group Study. 9 38
15493038 2004
12
Clonotypic IgM V/D/J sequence analysis in Waldenstrom macroglobulinemia suggests an unusual B-cell origin and an expansion of polyclonal B cells in peripheral blood. 9 38
14764523 2004
13
Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 9 38
15133473 2004
14
Lack of PAX5 rearrangements in lymphoplasmacytic lymphomas: reassessing the reported association with t(9;14). 9 38
15116325 2004
15
Expression of serotherapy target antigens in Waldenstrom's macroglobulinemia: therapeutic applications and considerations. 9 38
12720146 2003
16
Waldenström macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions. 9 38
12351413 2002
17
Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32). 9 38
10350329 1999
18
Peripheral neuropathy in IgM monoclonal gammopathy and Wäldenstrom's macroglobulinemia: a frequent complication in elderly males with low MAG-reactive serum monoclonal component. 9 38
7504399 1994
19
JL1 Antigen Expression on Bone Marrow Lymphoma Cells from Patients With Non-Hodgkin Lymphoma. 38
31432632 2020
20
Lymphoplasmacytic Lymphoma and Marginal Zone Lymphoma. 38
31229160 2019
21
Significance of urinary albumin excretion in patients with cast nephropathy
. 38
31232268 2019
22
Gamma-heavy chain monoclonal gammopathy with undetermined significance (MGUS). 38
31391403 2019
23
Analysis of 153,115 patients with hematological malignancies refines the spectrum of familial risk. 38
31395603 2019
24
Prolymphocytic transformation of lymphoplasmacytic lymphoma: an extremely unusual event. 38
30342054 2019
25
IgA-producing lymphoplasmacytic lymphoma carrying the chromosomal abnormality t(8;14). 38
31391404 2019
26
Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: a multicentric study of the Rete Ematologica Lombarda (REL) network. 38
31378966 2019
27
Disseminated strongyloidiasis complicated by alveolar hemorrhage, meningitis, and septic shock treated with albendazole and subcutaneous ivermectin. 38
31279610 2019
28
Cryoglobulinemia as a Possible Primer for TRALI: Report of a Case. 38
30657960 2019
29
Fine needle aspiration diagnosis of metastatic Leydig cell tumor. Report of a case and review of the literature. 38
31272604 2019
30
Acquired C1-inhibitor deficiency presenting with nephrotic syndrome. 38
31300605 2019
31
Bortezomib for the Treatment of Hematologic Malignancies: 15 Years Later. 38
30993606 2019
32
Rituximab use for lymphoplasmacytic lymphoma during continuous renal replacement therapy. 38
31271628 2019
33
CXCR4 mutations in lymphoplasmacytic lymphoma lead to altered CXCR4 expression. 38
30408147 2019
34
Uncommon lymphoplasmacytic lymphoma with IgA paraproteinemia: a challenging clinical diagnosis solved by MYD88 mutation analysis. 38
30426157 2019
35
Rare case of Bing-Neel syndrome treated successfully with ibrutinib. 38
31243026 2019
36
68Ga-pentixafor PET/CT for imaging of chemokine receptor-4 expression in Waldenström macroglobulinemia/lymphoplasmacytic lymphoma: comparison to 18F-FDG PET/CT. 38
31101745 2019
37
Generalized crystal-storing histiocytosis with diffuse large B-cell lymphoma and monoclonal gammopathy in a Chinese elderly woman: a case report. 38
31142285 2019
38
MYD88L265P mutated IgA lymphoplasmacytic lymphoma. 38
31107988 2019
39
Bortezomib-based chemotherapy can improve renal and tubular functions in patients with light chain-associated Fanconi syndrome. 38
30511139 2019
40
Updates in prognostication and treatment of Waldenström's macroglobulinemia. 38
31158330 2019
41
Biclonal presentation of lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia. 38
30846229 2019
42
MYD88, CARD11, and CD79B Oncogenic Mutations are Rare Events in the Indian Cohort of De Novo Nodal Diffuse Large B-Cell Lymphoma. 38
29734251 2019
43
Clinico-radiologic features and management of hematological tumors in the breast: a case series. 38
30229499 2019
44
Serum markers in the differential diagnosis of Waldenstrom macroglobulinemia and other IgM monoclonal gammopathies. 38
30485557 2019
45
Treatment modalities of non-Hodgkin lymphoma patients over 65 years of age: A two-center experience. 38
30924740 2019
46
Ibrutinib in the management of Waldenstrom macroglobulinemia. 38
29996737 2019
47
Waldenstrom macroglobulinemia retinopathy. Waldenstrom and eyes. 38
30679119 2019
48
Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management. 38
30328142 2019
49
CD13 expression in B cell malignancies is a hallmark of plasmacytic differentiation. 38
30198568 2019
50
Central Nervous System Involvement by Waldenstrom Macroglobulinemia: A Case Report of the Bing-Neel Syndrome. 38
31001436 2019

Variations for Lymphoplasmacytic Lymphoma

Cosmic variations for Lymphoplasmacytic 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 COSM5881377 NOTCH1 upper aerodigestive tract,mouth,other,dysplasia c.1699A>G p.I567V 9:136515687-136515687 0

Expression for Lymphoplasmacytic Lymphoma

Search GEO for disease gene expression data for Lymphoplasmacytic Lymphoma.

Pathways for Lymphoplasmacytic Lymphoma

Pathways related to Lymphoplasmacytic Lymphoma according to KEGG:

37
# Name Kegg Source Accession
1 Transcriptional misregulation in cancer hsa05202

Pathways related to Lymphoplasmacytic Lymphoma according to GeneCards Suite gene sharing:

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.25 ZAP70 MYD88 CXCR4 CXCL12 CD40LG CD40
2
Show member pathways
12.61 ZAP70 PTPRC CXCR4 CXCL12 CD40LG CD40
3
Show member pathways
12.5 ZAP70 MYD88 CXCL12 CD40LG CD40
4
Show member pathways
12.39 ZAP70 PTPRC IRF4 CD40LG
5 12.37 MIR494 MIR206 MIR155 CCND1
6
Show member pathways
12.23 PTPRC CXCR4 CXCL12 CCND1
7
Show member pathways
12.22 ZAP70 PTPRC CD40LG CD40
8 12.07 ZAP70 PTPRC PAX5 MYD88 IRF4 CXCR4
9 11.9 PTPRC MAG CD40LG CD40
10 11.7 PTPRC CXCR4 CXCL12
11 11.58 PTPRC CXCR4 CXCL12
12 11.56 PTPRC MS4A1 IRF4
13 11.56 ZAP70 MYD88 CXCL12 CD40LG CD40
14 11.32 MYD88 CD40LG CD40
15 11.13 ZAP70 PTPRC CD40LG CD40
16 11.1 ZAP70 CD40LG CD40
17 11.05 PTPRC CD40LG CD40
18 10.82 PTPRC PAX5 MS4A1 CXCR4 CD40LG CD40
19 10.4 MIR206 MAG

GO Terms for Lymphoplasmacytic Lymphoma

Cellular components related to Lymphoplasmacytic Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 9.1 PTPRC MS4A1 CXCR4 CXCL12 CD40LG CD40

Biological processes related to Lymphoplasmacytic Lymphoma according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 gene silencing by miRNA GO:0035195 9.92 MIR542 MIR494 MIR206 MIR184 MIR155
2 response to virus GO:0009615 9.71 MYD88 CXCR4 CXCL12
3 cellular response to mechanical stimulus GO:0071260 9.61 MYD88 MAG CD40
4 positive regulation of T cell differentiation GO:0045582 9.56 ZAP70 PTPRC
5 defense response to protozoan GO:0042832 9.55 IRF4 CD40
6 positive regulation of interleukin-2 biosynthetic process GO:0045086 9.51 PTPRC IRF4
7 T cell activation GO:0042110 9.5 ZAP70 PTPRC IRF4
8 positive thymic T cell selection GO:0045059 9.48 ZAP70 PTPRC
9 negative thymic T cell selection GO:0045060 9.46 ZAP70 PTPRC
10 positive regulation of isotype switching to IgG isotypes GO:0048304 9.4 PTPRC CD40
11 positive regulation of alpha-beta T cell proliferation GO:0046641 9.37 ZAP70 PTPRC
12 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.33 MIR494 MIR206 MIR155
13 regulation of immunoglobulin secretion GO:0051023 9.26 CD40LG CD40
14 regulation of B cell receptor signaling pathway GO:0050855 9.19 PAX5
15 B cell activation GO:0042113 9.13 ZAP70 MS4A1 CD40
16 B cell proliferation GO:0042100 8.92 PTPRC MS4A1 CD40LG CD40

Sources for Lymphoplasmacytic Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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