B-PLL
MCID: BCL005
MIFTS: 44

B Cell Prolymphocytic Leukemia (B-PLL)

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

Aliases & Classifications for B Cell Prolymphocytic Leukemia

MalaCards integrated aliases for B Cell Prolymphocytic Leukemia:

Name: B Cell Prolymphocytic Leukemia 53
Leukemia, Prolymphocytic, B-Cell 73
Leukemia, B Cell Prolymphocytic 53
B-Cell Prolymphocytic Leukemia 59
B-Pll 59

Characteristics:

Orphanet epidemiological data:

59
b-cell prolymphocytic leukemia
Age of onset: Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA86852
MESH via Orphanet 45 D054403
UMLS via Orphanet 74 C0475801
ICD10 via Orphanet 34 C91.3
ICD10 33 C91.3
UMLS 73 C0475801

Summaries for B Cell Prolymphocytic Leukemia

MalaCards based summary : B Cell Prolymphocytic Leukemia, also known as leukemia, prolymphocytic, b-cell, is related to prolymphocytic leukemia and mantle cell lymphoma. An important gene associated with B Cell Prolymphocytic Leukemia is CD5 (CD5 Molecule), and among its related pathways/superpathways are IL-2 Pathway and PI3K-Akt signaling pathway. The drugs Ofatumumab and Carmustine have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and t cells, and related phenotypes are hematopoietic system and digestive/alimentary

Wikipedia : 76 B-cell prolymphocytic leukemia is a more aggressive, but still treatable, form of leukemia. The... more...

Related Diseases for B Cell Prolymphocytic Leukemia

Diseases in the B Cell Prolymphocytic Leukemia family:

T-Cell Prolymphocytic Leukemia

Diseases related to B Cell Prolymphocytic Leukemia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 66)
# Related Disease Score Top Affiliating Genes
1 prolymphocytic leukemia 32.0 CD19 CD38 CD5 FCER2 MYC ZAP70
2 mantle cell lymphoma 29.6 CD19 CD5 MYC TP53
3 lymphocytic leukemia 29.3 CD38 CD5 FCER2 MYC TP53 ZAP70
4 b-cell lymphomas 29.2 CD5 FCER2 MS4A1 MYC TP53
5 leukemia, chronic lymphocytic 28.9 CD19 CD38 CD5 FCER2 IL2RA MS4A1
6 leukemia 10.8
7 bladder lymphoma 10.2 CD19 CD5
8 colon lymphoma 10.2 CD19 CD5
9 invasive malignant thymoma 10.2 CD19 CD38
10 non-secretory myeloma 10.2 CD19 CD38
11 gamma heavy chain disease 10.2 CD19 CD38
12 solitary osseous plasmacytoma 10.2 CD19 CD38
13 diffuse infiltrative lymphocytosis syndrome 10.2 CD19 CD38
14 anterior scleritis 10.2 CD19 CD5
15 leukemia, chronic lymphocytic 2 10.2
16 lymphoma 10.2
17 meningitis 10.2
18 leukemia, b-cell, chronic 10.2
19 lung lymphoma 10.1 CD19 CD5
20 monoclonal gammopathy of uncertain significance 10.1 CD19 CD38
21 toxic oil syndrome 10.1 FCER2 IL2RA
22 chromosome 13q14 deletion syndrome 10.1 CD38 ZAP70
23 cd45 deficiency 10.1 CD5 ZAP70
24 adult lymphoma 10.1 CD5 MYC
25 waldenstroem's macroglobulinemia 10.1 CD19 CD38 CD5
26 plasma protein metabolism disease 10.1 CD19 CD38 CD5
27 idiopathic neutropenia 10.1 FCER2 IL2RA
28 lymphoma, mucosa-associated lymphoid type 10.0 CD19 CD5 IGKC
29 lymphatic system cancer 10.0 CD19 CD5 MYC
30 lymphoma, hodgkin, classic 10.0 CD19 CD5
31 anemia, autoimmune hemolytic 10.0
32 hematopoietic stem cell transplantation 10.0
33 hemolytic anemia 10.0
34 pure red-cell aplasia 10.0
35 polyradiculoneuropathy 10.0
36 splenic diffuse red pulp small b-cell lymphoma 10.0
37 plasma cell neoplasm 10.0 CD19 CD38 MYC
38 necrotizing sialometaplasia 10.0 CD5 TP53
39 plasmacytoma 10.0 CD38 IGKC MYC
40 megaesophagus 9.9 MYC TP53
41 hypertrichosis universalis congenita, ambras type 9.9 MYC TP53
42 suppressor of tumorigenicity 3 9.9 MYC TP53
43 uterine corpus cancer 9.9 MYC TP53
44 t-cell prolymphocytic leukemia 9.9 MS4A1 MYC
45 plasmablastic lymphoma 9.9 CD38 MS4A1 MYC
46 cerebral primitive neuroectodermal tumor 9.9 MYC TP53
47 medullomyoblastoma 9.9 MYC TP53
48 follicular lymphoma 9.9 FCER2 MS4A1 MYC
49 childhood medulloblastoma 9.9 MYC TP53
50 fallopian tube carcinoma 9.9 MYC TP53

Graphical network of the top 20 diseases related to B Cell Prolymphocytic Leukemia:



Diseases related to B Cell Prolymphocytic Leukemia

Symptoms & Phenotypes for B Cell Prolymphocytic Leukemia

MGI Mouse Phenotypes related to B Cell Prolymphocytic Leukemia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.61 CD19 CD38 CD5 FCER2 IL2RA MS4A1
2 digestive/alimentary MP:0005381 9.55 CD19 FCER2 IL2RA MYC TP53
3 immune system MP:0005387 9.28 CD19 CD38 CD5 FCER2 IL2RA MS4A1

Drugs & Therapeutics for B Cell Prolymphocytic Leukemia

Drugs for B Cell Prolymphocytic Leukemia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 48)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ofatumumab Approved Phase 1, Phase 2 679818-59-8 6918251
2
Carmustine Approved, Investigational Phase 2 154-93-8 2578
3
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 6055-19-2, 50-18-0 2907
4
Fludarabine Approved Phase 2,Phase 1 75607-67-9, 21679-14-1 30751
5
Melphalan Approved Phase 2 148-82-3 4053 460612
6
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
7
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
8
Etoposide Approved Phase 2,Phase 1 33419-42-0 36462
9
Mycophenolic acid Approved Phase 2 24280-93-1 446541
10
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
11
Vidarabine Approved, Investigational Phase 2,Phase 1 24356-66-9 32326 21704
12
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
13
rituximab Approved Phase 1, Phase 2,Phase 2 174722-31-7 10201696
14
Obinutuzumab Approved, Investigational Phase 1, Phase 2 949142-50-1
15 Immunoglobulins Phase 1, Phase 2,Phase 2
16 Antibodies, Monoclonal Phase 1, Phase 2,Phase 2
17 Antibodies Phase 1, Phase 2,Phase 2
18 Immunologic Factors Phase 1, Phase 2,Phase 2
19 Antirheumatic Agents Phase 2,Phase 1
20 Antimetabolites, Antineoplastic Phase 2,Phase 1
21 Immunosuppressive Agents Phase 2,Phase 1
22 Antibiotics, Antitubercular Phase 2
23 Anti-Infective Agents Phase 2,Phase 1
24 Topoisomerase Inhibitors Phase 2,Phase 1
25 Antimetabolites Phase 2,Phase 1
26 Nitrogen Mustard Compounds Phase 2
27 phenylalanine Phase 2
28 Etoposide phosphate Phase 2,Phase 1
29 Alkylating Agents Phase 2,Phase 1
30 Vidarabine Phosphate Phase 2,Phase 1
31 Antineoplastic Agents, Alkylating Phase 2,Phase 1
32 Antineoplastic Agents, Phytogenic Phase 2,Phase 1
33 Antimitotic Agents Phase 2,Phase 1
34 Antitubercular Agents Phase 2
35 Keratolytic Agents Phase 2,Phase 1
36 alanine Phase 2
37 Calcineurin Inhibitors Phase 2
38 Podophyllotoxin Phase 2,Phase 1 518-28-5
39 Anti-Bacterial Agents Phase 2,Phase 1
40 Dermatologic Agents Phase 2,Phase 1
41 Antiviral Agents Phase 2,Phase 1
42 Cyclosporins Phase 2
43 Antifungal Agents Phase 2
44 tyrosine Phase 2
45
Lenalidomide Approved Phase 1 191732-72-6 216326
46 Bendamustine Hydrochloride Phase 1
47 Angiogenesis Modulating Agents Phase 1
48 Angiogenesis Inhibitors Phase 1

Interventional clinical trials:

(show all 17)
# Name Status NCT ID Phase Drugs
1 Ofatumumab and Dinaciclib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or B-Cell Prolymphocytic Leukemia Completed NCT01515176 Phase 1, Phase 2 Dinaciclib
2 Autologous Peripheral Blood Stem Cell Transplant Followed by Donor Bone Marrow Transplant in Treating Patients With High-Risk Hodgkin Lymphoma, Non-Hodgkin Lymphoma, Multiple Myeloma, or Chronic Lymphocytic Leukemia Completed NCT01008462 Phase 2 Carmustine;Cyclophosphamide;Cytarabine;Etoposide;Fludarabine Phosphate;Melphalan;Mycophenolate Mofetil;Tacrolimus
3 Fludarabine Phosphate and Total-Body Irradiation Before Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Chronic Lymphocytic Leukemia or Small Lymphocytic Leukemia Completed NCT00060424 Phase 2 Cyclosporine;Fludarabine Phosphate;Mycophenolate Mofetil
4 PCAR-119 Bridge Immunotherapy Prior to Stem Cell Transplant in Treating Patients With CD19 Positive Leukemia and Lymphoma Recruiting NCT02892695 Phase 1, Phase 2
5 Study Evaluating the Efficacy and Safety of PCAR-019 in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma Recruiting NCT02851589 Phase 1, Phase 2
6 CAR-T Cell Immunotherapy in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma Recruiting NCT02819583 Phase 1, Phase 2
7 Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma Recruiting NCT03010358 Phase 1, Phase 2 Entospletinib
8 PCI-32765 (Ibrutinib) in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or B-cell Prolymphocytic Leukemia Active, not recruiting NCT01589302 Phase 2 ibrutinib
9 Fludarabine, Cyclophosphamide, and Rituximab in Treating Patients With Prolymphocytic Leukemia Terminated NCT00281931 Phase 2 cyclophosphamide;fludarabine phosphate
10 Pharmacokinetic (PK) and Pharmacodynamic (PD) Study of Nelarabine in Patients With Relapsed/Refractory Lymphoid Malignancies Completed NCT01094860 Phase 1 Nelarabine
11 Cellular Immunotherapy Following Chemotherapy in Treating Patients With Recurrent Non-Hodgkin Lymphomas, Chronic Lymphocytic Leukemia or B-Cell Prolymphocytic Leukemia Recruiting NCT02153580 Phase 1 cyclophosphamide;Bendamustine Hydrochloride;Etoposide;Fludarabine Phosphate
12 Pevonedistat and Ibrutinib in Treating Participants With Relapsed or Refractory Chronic Lymphocytic Leukemia or Non-Hodgkin Lymphoma Recruiting NCT03479268 Phase 1 Ibrutinib;Pevonedistat
13 Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic Malignancies Recruiting NCT02924402 Phase 1
14 Lenalidomide and Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma Active, not recruiting NCT01886859 Phase 1 Ibrutinib;Lenalidomide
15 Alvespimycin Hydrochloride in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or B-Cell Prolymphocytic Leukemia Terminated NCT01126502 Phase 1 alvespimycin hydrochloride
16 Phase 1 Study Evaluating ZEN003365 in Relapsed/Refractory Lymphoproliferative Malignancies or Relapsed/Refractory AML Withdrawn NCT02238522 Phase 1 ZEN003365
17 Registry of the German CLL Study Group Recruiting NCT02863692

Search NIH Clinical Center for B Cell Prolymphocytic Leukemia

Genetic Tests for B Cell Prolymphocytic Leukemia

Anatomical Context for B Cell Prolymphocytic Leukemia

MalaCards organs/tissues related to B Cell Prolymphocytic Leukemia:

41
B Cells, Bone, T Cells, Myeloid, Bone Marrow, Lung, Colon

Publications for B Cell Prolymphocytic Leukemia

Articles related to B Cell Prolymphocytic Leukemia:

(show all 40)
# Title Authors Year
1
A Novel Treatment With Obinutuzumab-Chlorambucil in a Patient With B-Cell Prolymphocytic Leukemia: A Case Report and Review of the Literature. ( 30038912 )
2018
2
Auer Rod-Like Inclusions in B-cell prolymphocytic leukemia. ( 30238921 )
2018
3
Sequential Kinase Inhibition (Idelalisib/Ibrutinib) Induces Clinical Remission in B-Cell Prolymphocytic Leukemia Harboring a 17p Deletion. ( 28819574 )
2017
4
A 80-Year-Old Woman with B-Cell Prolymphocytic Leukemia. ( 28435655 )
2017
5
Development of B-cell prolymphocytic leukemia in a patient with splenic diffuse red pulp small B-cell lymphoma. ( 29199492 )
2017
6
Ibrutinib therapy is effective in B-cell prolymphocytic leukemia exhibiting MYC aberrations. ( 28695755 )
2017
7
PEITC in End-Stage B-Cell Prolymphocytic Leukemia: Case Report of Possible Sensitization to Salvage R-CHOP. ( 27168399 )
2016
8
Successful allogeneic hematopoietic stem cell transplantation with reduced-intensity conditioning for B-cell prolymphocytic leukemia in partial remission. ( 24470149 )
2014
9
An unusual cause of delirium and debility: refractory hypercalcemia in a man with B-cell prolymphocytic leukemia. ( 25333559 )
2014
10
Aberrations of MYC are a common event in B-cell prolymphocytic leukemia. ( 25125625 )
2014
11
B-cell prolymphocytic leukemia carrying t(8;14)(q24;q32), associated with both autoimmune hemolytic anemia and pure red cell aplasia. ( 25501113 )
2014
12
B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma. ( 24891323 )
2014
13
Smudge cells following treatment with pentostatin in a patient with B-cell prolymphocytic leukemia. ( 24049774 )
2013
14
De Novo B-Cell Prolymphocytic Leukemia with Central Nervous System Involvement. ( 24047478 )
2013
15
[CD5-positive B-cell prolymphocytic leukemia]. ( 20134145 )
2010
16
B-cell prolymphocytic leukemia and chronic lymphocytic leukemia have distinctive gene expression signatures. ( 19641528 )
2009
17
The first case of de novo B-cell prolymphocytic leukemia with central nervous system involvement: description of an unreported complication. ( 18929411 )
2009
18
Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia. ( 16997373 )
2007
19
IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). ( 16642047 )
2006
20
Allogeneic peripheral stem-cell transplantation with reduced-intensity conditioning regimen in refractory primary B-cell prolymphocytic leukemia: a long-term follow-up. ( 15880130 )
2005
21
Translocation (8;14)(q24;q32) as the sole cytogenetic abnormality in B-cell prolymphocytic leukemia. ( 15066324 )
2004
22
Successful treatment of B-cell prolymphocytic leukemia with monoclonal anti-CD20 antibody. ( 15060751 )
2004
23
Level of CD 20-expression and efficacy of rituximab treatment in patients with resistant or relapsing B-cell prolymphocytic leukemia and B-cell chronic lymphocytic leukemia. ( 11908720 )
2002
24
Frequent deletions at 11q23 and 13q14 in B cell prolymphocytic leukemia (B-PLL). ( 10720137 )
2000
25
B-cell prolymphocytic leukemia: a survey of 35 patients emphasizing heterogeneity, prognostic factors and evidence for a group with an indolent course. ( 10194135 )
1999
26
Leukemic pleural effusion in B-cell prolymphocytic leukemia. ( 9924927 )
1998
27
Cytogenetic abnormalities in three patients with B-cell prolymphocytic leukemia. ( 9595043 )
1998
28
5' region and exon 7 mutations of the TP53 gene in two cases of B-cell prolymphocytic leukemia. ( 9844609 )
1998
29
Leukemic meningitis in a patient with B-cell prolymphocytic leukemia. ( 9299880 )
1997
30
Major activity of cladribine in patients with de novo B-cell prolymphocytic leukemia. ( 8996122 )
1997
31
p53 abnormalities in B-cell prolymphocytic leukemia. ( 9058723 )
1997
32
Leukemic meningitis in B-cell prolymphocytic leukemia. A clinical, pathologic, and ultrastructural case study and a review of the literature. ( 7850707 )
1995
33
B-cell prolymphocytic leukemia expressing discordant myeloid-associated antigens in simultaneous specimens from bone marrow and peripheral blood. ( 8556956 )
1995
34
B-cell prolymphocytic leukemia expressing CD13 antigen. ( 7524729 )
1994
35
[Leukemic B-cell colony formation in patients with B-cell prolymphocytic leukemia]. ( 8479081 )
1993
36
Rapid response of B-cell prolymphocytic leukemia to 2-chlorodeoxyadenosine. ( 1357112 )
1992
37
Cytogenetic studies on prolymphocytic leukemia. 1. B-cell prolymphocytic leukemia. ( 3509770 )
1987
38
Fulminant leukemic polyradiculoneuropathy in a case of B-cell prolymphocytic leukemia. A clinicopathologic report. ( 3040217 )
1987
39
Interferon response in B-cell prolymphocytic leukemia. ( 3494471 )
1987
40
Chromosome abnormalities in B-cell prolymphocytic leukemia: a study of nine cases. ( 6603259 )
1983

Variations for B Cell Prolymphocytic Leukemia

Expression for B Cell Prolymphocytic Leukemia

Search GEO for disease gene expression data for B Cell Prolymphocytic Leukemia.

Pathways for B Cell Prolymphocytic Leukemia

Pathways related to B Cell Prolymphocytic Leukemia according to GeneCards Suite gene sharing:

(show all 23)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.84 CD19 FCER2 IL2RA MYC TP53
2
Show member pathways
12.71 CD19 IL2RA MYC TP53
3 12.22 CD19 CD38 IL2RA ZAP70
4 12.16 IL2RA MYC TP53
5
Show member pathways
12.01 FCER2 IGKC MYC TP53
6
Show member pathways
11.94 IL2RA MYC TP53
7 11.86 CD19 FCER2 MYC TP53
8 11.84 IL2RA MYC TP53
9 11.67 FCER2 MYC TP53
10 11.62 CD19 CD38 CD5 IL2RA MS4A1
11 11.41 CD19 CD38 MS4A1
12
Show member pathways
11.27 IL2RA MYC
13 11.27 MYC TP53
14 11.24 MYC TP53
15 11.21 MYC TP53
16 11.13 CD19 ZAP70
17 11.09 CD19 CD38 CD5 FCER2 IL2RA MS4A1
18 11.08 CD19 CD38 CD5 FCER2 MS4A1
19 11.07 MYC TP53
20 11.05 IL2RA ZAP70
21 10.97 MYC TP53
22 10.84 MYC TP53
23
Show member pathways
10.82 MYC TP53

GO Terms for B Cell Prolymphocytic Leukemia

Cellular components related to B Cell Prolymphocytic Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.56 CD19 CD38 CD5 FCER2 IGKC IL2RA
2 external side of plasma membrane GO:0009897 9.1 CD19 CD5 FCER2 IGKC IL2RA MS4A1

Biological processes related to B Cell Prolymphocytic Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of fibroblast proliferation GO:0048147 9.43 MYC TP53
2 B cell receptor signaling pathway GO:0050853 9.43 CD19 CD38 IGKC
3 B cell activation GO:0042113 9.4 MS4A1 ZAP70
4 response to gamma radiation GO:0010332 9.37 MYC TP53
5 Notch signaling pathway GO:0007219 9.33 FCER2 IL2RA MYC
6 positive regulation of T cell differentiation GO:0045582 9.32 IL2RA ZAP70
7 cell proliferation GO:0008283 9.26 CD5 IL2RA MYC TP53
8 cytokine-mediated signaling pathway GO:0019221 8.92 FCER2 IL2RA MYC TP53

Sources for B Cell Prolymphocytic Leukemia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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