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WM
MCID: LYM012
MIFTS: 61

Lymphoplasmacytic Lymphoma (WM)

Categories: Blood diseases, Cancer diseases, Neuronal diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Lymphoplasmacytic Lymphoma

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MalaCards integrated aliases for Lymphoplasmacytic Lymphoma:

Name: Lymphoplasmacytic Lymphoma 12 52 36 15 37
Waldenstrom Macroglobulinemia 12 52 25 58 29 54 43 39 17 71
Macroglobulinemia of Waldenstrom 52 25
Malignant Lymphoma - Lymphoplasmacytic 71
Waldenstrom's Macroglobulinaemia 52
Waldenström's Macroglobulinemia 74
Waldenstrom's Macroglobulinemia 25
Waldenström Macroglobulinemia 25
Waldenstrom's Syndrome 52
Wm 25

Characteristics:

Orphanet epidemiological data:

58
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:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Neuronal diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare neurological diseases
Rare haematological diseases


Sources

Summaries for Lymphoplasmacytic Lymphoma

About this section
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 MicroRNAs in cancer. The drugs Cyclophosphamide and Lenograstim 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 : 52 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 : 36 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 : 74 Waldenstrom's macroglobulinemia (WM), is a type of cancer affecting two types of B cells:... more...

Sources

Related Diseases for Lymphoplasmacytic Lymphoma

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

(show top 50) (show all 501)
# Related Disease Score Top Affiliating Genes
1 waldenstroem's macroglobulinemia 34.0 MYD88 MAG CXCR4 CD40LG
2 macroglobulinemia 31.9 PAX5 MYD88 CXCR4 CXCL12 CD40LG
3 lymphoma 31.6 PAX5 MYD88 MS4A1 IRF4 CXCR4 CD40LG
4 marginal zone b-cell lymphoma 31.2 PAX5 MYD88 IRF4 CD40LG CCND1
5 diffuse large b-cell lymphoma 31.0 PAX5 MYD88 MIR155 IRF4 CCND1
6 mononeuritis multiplex 31.0 MAG CD40LG
7 gamma heavy chain disease 31.0 MYD88 CD40LG
8 heavy chain disease 31.0 MYD88 CD40LG
9 whim syndrome 31.0 CXCR4 CXCL12 ACKR3
10 lymphoma, hodgkin, classic 30.9 PAX5 MS4A1 MIR155 IRF4
11 monoclonal paraproteinemia 30.9 PAX5 CD40LG
12 cll/sll 30.8 ZAP70 PAX5 CCND1
13 agammaglobulinemia, x-linked 30.8 CXCR4 CXCL12 CD40LG
14 leukemia, acute myeloid 30.8 PAX5 MIR184 MIR155 CXCR4 CXCL12 CCND1
15 central nervous system lymphoma 30.7 PAX5 MYD88 CXCR4 CXCL12
16 combined t cell and b cell immunodeficiency 30.7 ZAP70 IRF4 CD40LG
17 plasmacytoma 30.7 PAX5 IRF4 IGH CXCL12 CCND1
18 b-cell lymphoma 30.7 ZAP70 PAX5 MYD88 MS4A1 MIR155 IRF4
19 lymphocytic leukemia 30.7 ZAP70 PAX5 MYD88 MS4A1 MIR155 CD40LG
20 polyradiculoneuropathy 30.7 MAG CXCR4 CXCL12 CD40LG
21 monoclonal gammopathy of uncertain significance 30.7 MYD88 MAG CD40LG CCND1
22 plasma cell neoplasm 30.6 PAX5 IRF4 CD40LG CCND1
23 peripheral t-cell lymphoma 30.6 PAX5 MS4A1 CXCR4 CCND1
24 prolymphocytic leukemia 30.5 ZAP70 MS4A1 CCND1
25 myeloma, multiple 30.5 PAX5 MIR155 IRF4 CXCR4 CXCL12 CD40LG
26 follicular lymphoma 30.5 PAX5 MS4A1 MIR155 IRF4 IGH CCND1
27 plasma cell leukemia 30.5 IRF4 CCND1
28 extramedullary plasmacytoma 30.4 IRF4 CCND1
29 severe combined immunodeficiency 30.3 ZAP70 IRF4 CXCR4 CXCL12
30 lymphoma, mucosa-associated lymphoid type 30.3 ZAP70 PAX5 MYD88 IRF4 IGH CD40LG
31 mature b-cell neoplasm 30.3 PAX5 MIR155 IRF4 CD40LG CCND1
32 burkitt lymphoma 30.2 PAX5 MS4A1 MIR155 IRF4 CD40LG CCND1
33 lymphoma, non-hodgkin, familial 30.1 PAX5 MYD88 MS4A1 MIR155 IRF4 IGH
34 leukemia, chronic lymphocytic 30.0 ZAP70 PAX5 MYD88 MS4A1 MIR155 IRF4
35 leukemia, acute lymphoblastic 30.0 ZAP70 PAX5 MIR155 IRF4 IGH CXCR4
36 systemic lupus erythematosus 29.9 MYD88 MIR494 MIR184 MIR155 IRF4 CXCL12
37 mantle cell lymphoma 29.9 PAX5 MS4A1 MIR155 IGH CXCR4 CXCL12
38 lymphoplasmacytic lymphoma without igm production 12.4
39 weill-marchesani syndrome 12.0
40 macroglobulinemia, waldenstrom 1 11.9
41 tibia, hypoplasia or aplasia of, with polydactyly 11.5
42 williams-beuren syndrome 11.5
43 microspherophakia and/or megalocornea, with ectopia lentis and with or without secondary glaucoma 11.2
44 weill-marchesani syndrome 2 11.2
45 weill-marchesani syndrome 3 11.2
46 nodular lymphocyte predominant hodgkin lymphoma 10.6 PAX5 CCND1
47 intraocular lymphoma 10.6 PAX5 CXCR4 CXCL12
48 osteosclerotic myeloma 10.6 MAG CD40LG
49 aids dementia complex 10.6 CXCR4 CXCL12
50 primary cutaneous diffuse large b-cell lymphoma, leg type 10.6 MYD88 IRF4

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



Diseases related to Lymphoplasmacytic Lymphoma
Sources

Symptoms & Phenotypes for Lymphoplasmacytic Lymphoma

About this section

Human phenotypes related to Lymphoplasmacytic Lymphoma:

58 31 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 leukemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001909
2 lymphoma 58 31 hallmark (90%) Very frequent (99-80%) HP:0002665
3 monoclonal immunoglobulin m proteinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0005508
4 respiratory insufficiency 58 31 frequent (33%) Frequent (79-30%) HP:0002093
5 hypercoagulability 58 31 frequent (33%) Frequent (79-30%) HP:0100724
6 pallor 58 31 frequent (33%) Frequent (79-30%) HP:0000980
7 gingival bleeding 58 31 frequent (33%) Frequent (79-30%) HP:0000225
8 vertigo 58 31 frequent (33%) Frequent (79-30%) HP:0002321
9 abnormality of neutrophils 58 31 frequent (33%) Frequent (79-30%) HP:0001874
10 normocytic anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001897
11 ataxia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001251
12 hearing impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0000365
13 splenomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0001744
14 hepatomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0002240
15 malabsorption 58 31 occasional (7.5%) Occasional (29-5%) HP:0002024
16 reduced consciousness/confusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0004372
17 fatigue 58 31 occasional (7.5%) Occasional (29-5%) HP:0012378
18 renal insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0000083
19 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
20 cranial nerve paralysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006824
21 peripheral neuropathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0009830
22 congestive heart failure 58 31 occasional (7.5%) Occasional (29-5%) HP:0001635
23 urticaria 58 31 occasional (7.5%) Occasional (29-5%) HP:0001025
24 pulmonary infiltrates 58 31 occasional (7.5%) Occasional (29-5%) HP:0002113
25 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
26 memory impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002354
27 anorexia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002039
28 migraine 58 31 occasional (7.5%) Occasional (29-5%) HP:0002076
29 proptosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000520
30 purpura 58 31 occasional (7.5%) Occasional (29-5%) HP:0000979
31 recurrent infections 58 31 occasional (7.5%) Occasional (29-5%) HP:0002719
32 diarrhea 58 31 occasional (7.5%) Occasional (29-5%) HP:0002014
33 epistaxis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000421
34 stroke 58 31 occasional (7.5%) Occasional (29-5%) HP:0001297
35 vasculitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002633
36 periorbital edema 58 31 occasional (7.5%) Occasional (29-5%) HP:0100539
37 lymphadenopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0002716
38 cutis marmorata 58 31 occasional (7.5%) Occasional (29-5%) HP:0000965
39 retinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0000573
40 pleural effusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0002202
41 elevated erythrocyte sedimentation rate 58 31 occasional (7.5%) Occasional (29-5%) HP:0003565
42 cryoglobulinemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0100778
43 multifocal epileptiform discharges 58 31 occasional (7.5%) Occasional (29-5%) HP:0010841
44 pedal edema 31 occasional (7.5%) HP:0010741
45 abnormal retinal vascular morphology 31 occasional (7.5%) HP:0008046
46 abnormality of the retinal vasculature 58 Occasional (29-5%)
47 edema of the lower limbs 58 Occasional (29-5%)

MGI Mouse Phenotypes related to Lymphoplasmacytic Lymphoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 ACKR3 CCND1 CD40LG CXCL12 CXCR4 IRF4
2 immune system MP:0005387 9.36 ACKR3 CCND1 CD40LG CXCL12 CXCR4 IRF4
Sources

Drugs & Therapeutics for Lymphoplasmacytic Lymphoma

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

(show top 50) (show all 319)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
2
Lenograstim Approved, Investigational Phase 4 135968-09-1
3
Thiotepa Approved, Investigational Phase 4 52-24-4 5453
4 Alkylating Agents Phase 4
5 Orange Approved Phase 3
6
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
7
Chlorambucil Approved Phase 3 305-03-3 2708
8
Etoposide Approved Phase 2, Phase 3 33419-42-0 36462
9
Ondansetron Approved Phase 3 99614-02-5 4595
10
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
11
Carboplatin Approved Phase 2, Phase 3 41575-94-4 10339178 498142 38904
12
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
13
Morphine Approved, Investigational Phase 3 57-27-2 5288826
14
Darbepoetin alfa Approved, Investigational Phase 3 209810-58-2, 11096-26-7
15
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
16
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 468682 2826718
17
Piperacillin Approved Phase 3 66258-76-2 43672
18
Tazobactam Approved Phase 3 89786-04-9 123630
19
Vancomycin Approved Phase 3 1404-90-6 441141 14969
20
Amphotericin B Approved, Investigational Phase 3 1397-89-3 14956 5280965
21
Idelalisib Approved Phase 3 870281-82-6
22
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
23
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
24 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
25 Anesthetics, Dissociative Phase 3
26 Antiemetics Phase 3
27 Gastrointestinal Agents Phase 3
28 Autonomic Agents Phase 3
29 Etoposide phosphate Phase 2, Phase 3
30 Neurotransmitter Agents Phase 3
31 Hematinics Phase 3
32 Central Nervous System Depressants Phase 3
33 Tranquilizing Agents Phase 3
34 Psychotropic Drugs Phase 3
35 Antiparasitic Agents Phase 3
36 Antiprotozoal Agents Phase 3
37 Adjuvants, Immunologic Phase 3
38 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
39 Analgesics Phase 3
40 Liver Extracts Phase 3
41 Cola Phase 3
42 Antipsychotic Agents Phase 3
43 Analgesics, Opioid Phase 3
44 Antipruritics Phase 3
45 Anti-Anxiety Agents Phase 3
46 Serotonin Agents Phase 3
47 Serotonin Antagonists Phase 3
48 Epoetin alfa Phase 3 113427-24-0
49 Narcotics Phase 3
50 Anesthetics Phase 3

Interventional clinical trials:

(show top 50) (show all 494)
# 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 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
16 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
17 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
18 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
19 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
20 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
21 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
22 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
23 Vancomycin Versus Placebo in Persistently Febrile Granulocytopenic Patients Given Piperacillin/Tazobactam Completed NCT00003805 Phase 3 piperacillin sodium;piperacillin-tazobactam;tazobactam sodium;vancomycin
24 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
25 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 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 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
29 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
30 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
31 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
32 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
33 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
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 Clinical Trial of the mTor Inhibitor RAD001 (Everolimus) in Combination With Lenalidomide (Revlimid) for Patients With Relapsed or Refractory Lymphoid Malignancy Unknown status NCT01075321 Phase 1, Phase 2 everolimus;lenalidomide
45 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
46 Myeloblative Therapy With Autologous Hematopoietic Stem Cell Transplantation in Patients With Multiple Myeloma and B-Cell Malignancies Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
47 A Phase II Study to Investigate Biological Correlates of Clinical Response to Panobinostat in Haematological Malignancy Unknown status NCT01658241 Phase 2 panobinostat
48 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
49 A Phase II Study of Perifosine in Patients With Relapsed/Refractory Waldenstrom's Macroglobulinemia Completed NCT00422656 Phase 2 Perifosine
50 Phase II Study of Combination Rituxan (Rituximab, Mabthera) and Fludarabine Therapy in Lymphoplasmacytic Lymphoma (Waldenstrom's Macroglobulinemia) Completed NCT00020800 Phase 2 fludarabine phosphate

Search NIH Clinical Center for Lymphoplasmacytic Lymphoma

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Chlorambucil
Cyclophosphamide
Ibrutinib
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.
Stem-cell-based therapeutic approaches for Lymphoplasmacytic Lymphoma:
Hematopoietic stem cells for treatment of hematologic malignancies
Blood stem cell transplantation for hematological diseases
Embryonic/Adult Cultured Cells Related to Lymphoplasmacytic Lymphoma:
Peripheral blood-derived hematopoietic stem cells (family)
Bone marrow-derived hematopoietic stem cells

Cochrane evidence based reviews: waldenstrom macroglobulinemia

Sources

Genetic Tests for Lymphoplasmacytic Lymphoma

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Genetic tests related to Lymphoplasmacytic Lymphoma:

# Genetic test Affiliating Genes
1 Waldenstrom Macroglobulinemia 29 MYD88
Sources

Anatomical Context for Lymphoplasmacytic Lymphoma

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MalaCards organs/tissues related to Lymphoplasmacytic Lymphoma:

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

Publications for Lymphoplasmacytic Lymphoma

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Articles related to Lymphoplasmacytic Lymphoma:

(show top 50) (show all 875)
# Title Authors PMID Year
1
microRNA expression in the biology, prognosis, and therapy of Waldenström macroglobulinemia. 46
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. 54 61
18815290 2008
3
The HMG-CoA inhibitor, simvastatin, triggers in vitro anti-tumour effect and decreases IgM secretion in Waldenstrom macroglobulinaemia. 54 61
18537966 2008
4
Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. 54 61
18452095 2008
5
Genomewide linkage screen for Waldenstrom macroglobulinemia susceptibility loci in high-risk families. 54 61
16960805 2006
6
Expression of inhibitor of apoptosis proteins in B-cell non-Hodgkin and Hodgkin lymphomas. 54 61
16983704 2006
7
Translocation t(9;14)(p13;q32) in cases of splenic marginal zone lymphoma. 54 61
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. 54 61
16280661 2005
9
Pentostatin/cyclophosphamide with or without rituximab: an effective regimen for patients with Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma. 54 61
16231851 2005
10
How we treat Waldenström's macroglobulinemia. 54 61
15642678 2005
11
Initial immunoglobulin M 'flare' after rituximab therapy in patients diagnosed with Waldenstrom macroglobulinemia: an Eastern Cooperative Oncology Group Study. 54 61
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. 54 61
14764523 2004
13
Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 54 61
15133473 2004
14
Lack of PAX5 rearrangements in lymphoplasmacytic lymphomas: reassessing the reported association with t(9;14). 54 61
15116325 2004
15
Expression of serotherapy target antigens in Waldenstrom's macroglobulinemia: therapeutic applications and considerations. 54 61
12720146 2003
16
Waldenström macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions. 54 61
12351413 2002
17
Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32). 54 61
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. 54 61
7504399 1994
19
Clinical, Laboratory, and Bone Marrow Findings of 31 Patients With Waldenström Macroglobulinemia. 61
31858758 2020
20
Lymphoplasmacytic lymphoma infiltrating both lacrimal glands in a patient with Waldenström's macroglobulinemia. 61
32016162 2020
21
Crystal-storing histiocytosis leading to the identification of IgG-kappa secreting lymphoplasmacytic lymphoma with crystalline nephropathy. 61
31883675 2020
22
A man in his 70s with neurological symptoms and progressive pain during cancer therapy. 61
32026856 2020
23
Health-related quality of life in Waldenstrom Macroglobulinemia and IgM-related disorders: A single institution experience. 61
31852014 2020
24
Hepatitis C Virus-associated Lymphoplasmacytic Lymphoma With Waldenström Macroglobulinemia: Response to Direct-acting Antiviral Therapy. 61
32033929 2020
25
JL1 Antigen Expression on Bone Marrow Lymphoma Cells from Patients With Non-Hodgkin Lymphoma. 61
31432632 2020
26
Treatment modalities of non-Hodgkin lymphoma patients over 65 years of age: A two-center experience. 61
30924740 2020
27
Lymphoplasmacytic lymphoma and Waldenström macroglobulinaemia: clinicopathological features and differential diagnosis. 61
31767130 2020
28
Bing-Neel syndrome presenting as isolated CNS lymphoplasmacytic lymphoma: A case report and review of the literature. 61
31862225 2020
29
A matched case-control study comparing features, treatment and outcomes between patients with non-IgM lymphoplasmacytic lymphoma and Waldenström macroglobulinemia. 61
31992103 2020
30
Diagnostic value of bone marrow core biopsy patterns in lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia and description of its mutational profiles by targeted NGS. 61
31980558 2020
31
Waldenstrom's macroglobulinemia in the era of immunotherapy. 61
31960730 2020
32
68Ga-Pentixafor PET/CT for Imaging of Chemokine Receptor 4 Expression in Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma: Comparison to 18F-FDG PET/CT. 61
31101745 2019
33
Exclusive B-cell phenotype of primary prostatic lymphomas. A potential role of chronic prostatitis. 61
31825109 2019
34
Updates in prognostication and treatment of Waldenström's macroglobulinemia. 61
31158330 2019
35
Deepening of response after completing rituximab-containing therapy in patients with Waldenstrom macroglobulinemia. 61
31868242 2019
36
Outcomes of bendamustine- or cyclophosphamide-based first-line chemotherapy in older patients with indolent B-cell lymphoma. 61
31849108 2019
37
What is new in the treatment of Waldenstrom macroglobulinemia? 61
31591468 2019
38
Incidental Lymphoplasmacytic Lymphoma Diagnosed Following Robotic-Assisted Laparoscopic Prostatectomy for Prostate Cancer. 61
31933596 2019
39
Severe nephritis as initial sign of Waldenström's macroglobulinemia. 61
31929868 2019
40
Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: A multicentric study of the Rete Ematologica Lombarda (REL) network. 61
31378966 2019
41
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-3. Lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia (LPL/WM). 61
31494831 2019
42
Human MYD88L265P is insufficient by itself to drive neoplastic transformation in mature mouse B cells. 61
31698464 2019
43
Clinicopathological Features and Outcomes in Lymphoma of Extraocular Muscles. 61
31577647 2019
44
MYD88L265P mutated IgA lymphoplasmacytic lymphoma. 61
31107988 2019
45
MYD88 L265P mutation analysis is a useful diagnostic adjunct for lymphoplasmacytic lymphoma with pleural effusion. 61
31556196 2019
46
Kidney diseases associated with Waldenström macroglobulinemia. 61
30380110 2019
47
Disseminated cytomegalovirus disease after bendamustine: a case report and analysis of circulating B- and T-cell subsets. 61
31640581 2019
48
Maculopathy in Patients with Monoclonal Gammopathy of Undetermined Significance. 61
31753810 2019
49
Development of Central Nervous System Vasculitis in a Patient with Waldenstrom Macroglobulinemia: A Rare Presentation with Poor Prognosis. 61
31824806 2019
50
Analysis of 153 115 patients with hematological malignancies refines the spectrum of familial risk. 61
31395603 2019
Sources

Variations for Lymphoplasmacytic Lymphoma

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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 COSM142567306 upper aerodigestive tract,mouth,other,dysplasia c.355C>T p.R119C 17:7675140-7675140 0
Sources

Expression for Lymphoplasmacytic Lymphoma

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Search GEO for disease gene expression data for Lymphoplasmacytic Lymphoma.
Sources

Pathways for Lymphoplasmacytic Lymphoma

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Pathways related to Lymphoplasmacytic Lymphoma according to KEGG:

36
# Name Kegg Source Accession
1 Transcriptional misregulation in cancer hsa05202

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

# Super pathways Score Top Affiliating Genes
1
MicroRNAs in cancer 36
12.11 MIR494 MIR206 MIR155 CCND1
2
NF-kappaB Signaling 4
11.68 ZAP70 PAX5 MYD88 IRF4 CXCR4 CXCL12
3
NF-kappa B signaling pathway 36
11.47 ZAP70 MYD88 CXCL12 CD40LG
4
B Cell Development Pathways 64
10.98 PAX5 MS4A1 CXCR4 CD40LG
5
Glial Cell Differentiation 1
10.16 MIR206 MAG
Sources

GO Terms for Lymphoplasmacytic Lymphoma

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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 9.02 MS4A1 CXCR4 CXCL12 CD40LG ACKR3

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.72 ZAP70 CXCR4 CXCL12 CD40LG ACKR3
2 cell chemotaxis GO:0060326 9.61 CXCR4 CXCL12 ACKR3
3 negative regulation of intrinsic apoptotic signaling pathway in response to DNA damage GO:1902230 9.48 CXCL12 ACKR3
4 detection of temperature stimulus involved in sensory perception of pain GO:0050965 9.46 CXCR4 CXCL12
5 detection of mechanical stimulus involved in sensory perception of pain GO:0050966 9.4 CXCR4 CXCL12
6 cellular response to oxidised low-density lipoprotein particle stimulus GO:0140052 9.37 MYD88 MIR155
7 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.33 MIR494 MIR206 MIR155
8 telencephalon cell migration GO:0022029 9.26 CXCR4 CXCL12
9 positive regulation of mesenchymal stem cell migration GO:1905322 8.96 CXCR4 ACKR3
10 response to ultrasound GO:1990478 8.62 CXCR4 CXCL12

Molecular functions related to Lymphoplasmacytic Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 C-X-C chemokine receptor activity GO:0016494 8.62 CXCR4 ACKR3
Sources

Sources for Lymphoplasmacytic 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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