B-PLL
MCID: BCL005
MIFTS: 39

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 20
B-Cell Prolymphocytic Leukemia 58 17
Leukemia, Prolymphocytic, B-Cell 70
Leukemia, B Cell Prolymphocytic 20
B-Pll 58

Characteristics:

Orphanet epidemiological data:

58
b-cell prolymphocytic leukemia
Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 32 C91.3
MESH via Orphanet 45 D054403
ICD10 via Orphanet 33 C91.3
UMLS via Orphanet 71 C0475801
Orphanet 58 ORPHA86852
UMLS 70 C0475801

Summaries for B Cell Prolymphocytic Leukemia

MalaCards based summary : B Cell Prolymphocytic Leukemia, also known as b-cell prolymphocytic leukemia, is related to anemia, autoimmune hemolytic and prolymphocytic leukemia. An important gene associated with B Cell Prolymphocytic Leukemia is CD38 (CD38 Molecule), and among its related pathways/superpathways are PI3K-Akt signaling pathway and Measles. The drugs Carmustine and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, t cells and myeloid, and related phenotypes are hematopoietic system and immune system

Wikipedia : 73 B-cell prolymphocytic leukemia, referred to as B-PLL, is a rare blood cancer. It is a more aggressive,... 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 110)
# Related Disease Score Top Affiliating Genes
1 anemia, autoimmune hemolytic 30.4 ZAP70 CD5 CD19
2 prolymphocytic leukemia 30.2 ZAP70 TP53 MYC IL2RA FCER2 CD5
3 t-cell prolymphocytic leukemia 29.7 MYC MS4A1
4 leukemia, chronic lymphocytic 2 29.4 ZAP70 MS4A1 FCER2 CD5 CD38
5 mantle cell lymphoma 29.1 TP53 MYC MS4A1 CD5 CD19
6 leukemia, acute lymphoblastic 28.9 ZAP70 TP53 MYC CD5 CD38 CD19
7 burkitt lymphoma 28.8 TP53 MYC MS4A1 FCER2 CD19
8 leukemia, chronic lymphocytic 28.5 ZAP70 TP53 MYC MS4A1 IL2RA FCER2
9 lymphoma 28.5 TP53 MYC MS4A1 IL2RA CD5 CD38
10 b-cell lymphoma 28.3 ZAP70 TP53 MYC MS4A1 FCER2 CD5
11 splenomegaly 10.6
12 leukemia 10.5
13 chromosomal triplication 10.4
14 meningitis 10.3
15 refractory hematologic cancer 10.2 CD38 CD19
16 mental retardation, autosomal dominant 36 10.2 CD38 CD19
17 deficiency anemia 10.2
18 lymphoproliferative syndrome 10.2
19 hemolytic anemia 10.2
20 invasive malignant thymoma 10.2 CD38 CD19
21 eye lymphoma 10.2 CD5 CD19
22 alpha chain disease 10.2 CD5 CD19
23 schuurs-hoeijmakers syndrome 10.2 CD38 CD19
24 extramedullary plasmacytoma 10.1 IGKC CD38
25 mulchandani-bhoj-conlin syndrome 10.1 CD38 CD19
26 spleen cancer 10.1 CD5 CD19
27 gallbladder lymphoma 10.1 FCER2 CD5
28 multicentric castleman disease 10.1 CD38 CD19
29 refractory hairy cell leukemia 10.1 IL2RA CD19
30 bacterial infectious disease 10.1
31 neutropenia 10.1
32 pure red-cell aplasia 10.1
33 polyradiculoneuropathy 10.1
34 herpes zoster 10.1
35 subacute delirium 10.1
36 47,xyy 10.1
37 splenic diffuse red pulp small b-cell lymphoma 10.1
38 disseminated eosinophilic collagen disease 10.1 IL2RA CD5
39 toxic oil syndrome 10.1 IL2RA FCER2
40 lymphoplasmacytic lymphoma 10.1 CD5 CD38 CD19
41 plasma protein metabolism disease 10.1 CD5 CD38 CD19
42 t cell deficiency 10.1 ZAP70 CD19
43 autoimmune disease of blood 10.1 CD5 CD19
44 idiopathic neutropenia 10.0 IL2RA FCER2
45 splenic disease 10.0 CD5 CD19
46 wells syndrome 10.0 TP53 IL2RA
47 primary cutaneous t-cell lymphoma 10.0 MS4A1 IL2RA
48 relapsed/refractory diffuse large b-cell lymphoma 9.9 MYC CD19
49 appendix lymphoma 9.9 MYC CD5
50 thrombocytopenia 9.9

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 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 top 50) (show all 52)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carmustine Approved, Investigational Phase 2 154-93-8 2578
2
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
3
Melphalan Approved Phase 2 148-82-3 4053 460612
4
Mycophenolic acid Approved Phase 2 24280-93-1 446541
5
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492 6473866
6
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
7
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
8
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
9
ofatumumab Approved Phase 1, Phase 2 679818-59-8 6918251
10
acetic acid Approved Phase 2 64-19-7 176
11
Piperazine Approved, Vet_approved Phase 2 110-85-0 4837
12
Mebendazole Approved, Vet_approved Phase 2 31431-39-7 4030
13
alemtuzumab Approved, Investigational Phase 2 216503-57-0
14
Obinutuzumab Approved, Investigational Phase 1, Phase 2 949142-50-1
15
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
16
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
17
rituximab Approved Phase 2 174722-31-7 10201696
18
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
19
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
20 Antibiotics, Antitubercular Phase 2
21 Antitubercular Agents Phase 2
22 Antiviral Agents Phase 2
23 Calcineurin Inhibitors Phase 2
24 Nitrogen Mustard Compounds Phase 2
25 Cyclosporins Phase 2
26 Antifungal Agents Phase 2
27 Anti-Bacterial Agents Phase 2
28 Anti-Infective Agents Phase 2
29
Retinol acetate Phase 2 127-47-9 10245972
30 DMP 777 Phase 2 157341-41-8
31 Piperazine citrate Phase 2
32 Immunoglobulins Phase 1, Phase 2
33 Antibodies Phase 1, Phase 2
34 Antibodies, Monoclonal Phase 1, Phase 2
35 Antirheumatic Agents Phase 2
36 Immunosuppressive Agents Phase 2
37 Alkylating Agents Phase 2
38 Immunologic Factors Phase 2
39 Antimetabolites Phase 2
40 Antineoplastic Agents, Immunological Phase 2
41
L-Alanine Nutraceutical Phase 2 56-41-7 5950
42
Etoposide Approved Phase 1 33419-42-0 36462
43
Lenalidomide Approved Phase 1 191732-72-6 216326
44
Pevonedistat Investigational Phase 1 905579-51-3
45 Tubulin Modulators Phase 1
46 Antimitotic Agents Phase 1
47 Etoposide phosphate Phase 1
48 Dermatologic Agents Phase 1
49 Podophyllotoxin Phase 1 518-28-5
50 Keratolytic Agents Phase 1

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 Study Evaluating the Efficacy and Safety of PCAR-019 in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma Unknown status NCT02851589 Phase 1, Phase 2
2 Study Evaluating the Efficacy and Safety of PCAR-019 in CD19 Positive Relapsed or Refractory Leukemia and Lymphoma Unknown status NCT02819583 Phase 1, Phase 2
3 PCAR-119 Bridge Immunotherapy Prior to Stem Cell Transplant in Treating Patients With CD19 Positive Relapsed or Refractory Leukemia and Lymphoma Unknown status NCT02892695 Phase 1, Phase 2
4 Sequential Autologous HCT / Nonmyeloablative Allogeneic HCT Using Related, HLA-Haploidentical Donors for Patients With High-Risk Lymphoma, Multiple Myeloma, or Chronic Lymphocytic Leukemia Completed NCT01008462 Phase 2 Carmustine;Cyclophosphamide;Cytarabine;Etoposide;Fludarabine Phosphate;Melphalan;Mycophenolate Mofetil;Tacrolimus
5 Allogeneic Hematopoietic Stem Cell Transplantation With Nonmyeloablative Conditioning for Patients With Chronic Lymphocytic Leukemia - A Multi-center Trial Completed NCT00060424 Phase 2 Cyclosporine;Fludarabine Phosphate;Mycophenolate Mofetil
6 Phase 1b/2 Study of Dinaciclib (SCH 727965) and Ofatumumab in Relapsed and Refractory CLL/SLL/B-PLL Completed NCT01515176 Phase 1, Phase 2 Dinaciclib
7 Letermovir for Cytomegalovirus Prophylaxis in Patients With Hematological Malignancies Treated With Alemtuzumab Recruiting NCT04312841 Phase 2 Letermovir
8 A Phase 2 Study of the Bruton's Tyrosine Kinase (Btk) Inhibitor, PCI-32765(Ibrutinib), in Relapsed and Refractory Patients With Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL) and B-cell Prolymphocytic Leukemia (B-PLL) Active, not recruiting NCT01589302 Phase 2 ibrutinib
9 A Phase I/II Study of Syk Inhibitor Entospletinib (GS-9973) in Combination With Obinutuzumab in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and B-Cell Malignancies Active, not recruiting NCT03010358 Phase 1, Phase 2 Entospletinib
10 Phase II Trial of Combined Immunochemotherapy With Fludarabine, Cyclophosphamide and Rituximab in Patients With B-PLL Terminated NCT00281931 Phase 2 cyclophosphamide;fludarabine phosphate
11 A Phase I Study of Pevonedistat (MLN4924, TAK924) in Combination With Ibrutinib in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma Recruiting NCT03479268 Phase 1 Ibrutinib;Pevonedistat
12 Phase I Study to Evaluate Cellular Immunotherapy Using Memory-Enriched T Cells Lentivirally Transduced to Express a CD19-Specific, Hinge-Optimized, CD28-Costimulatory Chimeric Receptor and a Truncated EGFR Following Lymphodepleting Chemotherapy in Adult Patients With CD19+ B-Cell Lymphoproliferative Neoplasms Recruiting NCT02153580 Phase 1 cyclophosphamide;Bendamustine Hydrochloride;Etoposide;Fludarabine Phosphate
13 A Dose Escalation Study of Ibrutinib With Lenalidomide for Relapsed and Refractory Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Active, not recruiting NCT01886859 Phase 1 Ibrutinib;Lenalidomide
14 A Phase I Study of the Hsp90 Inhibitor 17-DMAG (Alvespimycin) in Patients With Relapsed Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL), and B-cell Prolymphocytic Leukemia (B-PLL) Terminated NCT01126502 Phase 1 alvespimycin hydrochloride
15 Registry of the German CLL Study Group Long Term Follow-up of Patients With CLL, B-PLL, T-PLL, SLL,T or NK-LGL, HCL and Richter's Transformation 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:

40
Bone Marrow, T Cells, Myeloid, B Cells, Eye, Bone, Spleen

Publications for B Cell Prolymphocytic Leukemia

Articles related to B Cell Prolymphocytic Leukemia:

(show top 50) (show all 99)
# Title Authors PMID Year
1
Shikonin Derivatives from Onsoma visianii Decrease Expression of Phosphorylated STAT3 in Leukemia Cells and Exert Antitumor Activity. 61
33807148 2021
2
An Unusual Case of Prolymphocytic Leukemia Transformation in a Patient With Chronic Lymphocytic Leukemia. 61
33533282 2021
3
Effective upfront treatment with low-dose ibrutinib for a patient with B cell prolymphocytic leukemia. 61
31965420 2020
4
An illustrative case of B-cell prolymphocytic leukemia. 61
32883890 2020
5
Initial treatment of B-cell prolymphocytic leukemia with ibrutinib. 61
31951033 2020
6
B-cell prolymphocytic leukemia successfully treated with B-cell receptor antagonists, but resistant to venetoclax. 61
31713446 2020
7
A Novel t(10;22) Translocation Harboring an IGL Gene Deletion in a CLL Patient Transforming to B-PLL with 1q Gain. 61
32526732 2020
8
B-cell prolymphocytic leukemia has 3 subsets. 61
31751477 2019
9
Genetic characterization of B-cell prolymphocytic leukemia: a prognostic model involving MYC and TP53. 61
31527074 2019
10
Auer Rod-Like Inclusions in B-Cell Prolymphocytic Leukemia 61
30238921 2019
11
B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront. 61
31700732 2019
12
[High-dose methylprednisolone with Rituximab and fresh frozen plasma in the treatment of six patients with B-cell lymphoproliferative disorders harboring TP53 abnormalities]. 61
31207703 2019
13
Hot and Cold: A Concurrent Warm and Cold Autoimmune Hemolytic Anemia in B-cell Prolymphocytic Leukemia. 61
30947164 2019
14
Development of B-cell prolymphocytic leukemia in a patient with splenic diffuse red pulp small B-cell lymphoma. 61
29199492 2018
15
Ibrutinib therapy is effective in B-cell prolymphocytic leukemia exhibiting MYC aberrations. 61
28695755 2018
16
A Novel Treatment With Obinutuzumab-Chlorambucil in a Patient With B-Cell Prolymphocytic Leukemia: A Case Report and Review of the Literature. 61
30038912 2018
17
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment. 61
28324286 2017
18
A 80-Year-Old Woman with B-Cell Prolymphocytic Leukemia. 61
28435655 2017
19
Sequential Kinase Inhibition (Idelalisib/Ibrutinib) Induces Clinical Remission in B-Cell Prolymphocytic Leukemia Harboring a 17p Deletion. 61
28819574 2017
20
PEITC in End-Stage B-Cell Prolymphocytic Leukemia: Case Report of Possible Sensitization to Salvage R-CHOP. 61
27168399 2016
21
An unusual cause of delirium and debility: refractory hypercalcemia in a man with B-cell prolymphocytic leukemia. 61
25333559 2014
22
Aberrations of MYC are a common event in B-cell prolymphocytic leukemia. 61
25125625 2014
23
B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma. 61
24891323 2014
24
De novo B-cell prolymphocytic leukemia with central nervous system involvement. 61
24047478 2014
25
[The immunophenotypic characteristics of 260 patients with CD5 + B cell lymphoproliferative disorders]. 61
24759024 2014
26
Successful allogeneic hematopoietic stem cell transplantation with reduced-intensity conditioning for B-cell prolymphocytic leukemia in partial remission. 61
24470149 2014
27
B-cell prolymphocytic leukemia carrying t(8;14)(q24;q32), associated with both autoimmune hemolytic anemia and pure red cell aplasia. 61
25501113 2014
28
Smudge cells following treatment with pentostatin in a patient with B-cell prolymphocytic leukemia. 61
24049774 2013
29
[Clinical characteristics of 8 patients with B-cell prolymphocytic leukemia]. 61
23827118 2013
30
BRAF mutations in chronic lymphocytic leukemia. 61
23088640 2013
31
Splenic B-cell lymphomas with more than 55% prolymphocytes in blood: evidence for prolymphocytoid transformation. 61
22520947 2012
32
How I treat prolymphocytic leukemia. 61
22649104 2012
33
Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers. 61
21850989 2011
34
Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. 61
20558005 2011
35
[Successful treatment of B-cell prolymphocytic leukemia (B-PLL) with FCR-Lite (fludarabine, cyclophosphamide, rituximab) protocol]. 61
20656663 2010
36
[CD5-positive B-cell prolymphocytic leukemia]. 61
20134145 2010
37
B-cell prolymphocytic leukemia and chronic lymphocytic leukemia have distinctive gene expression signatures. 61
19641528 2009
38
The first case of de novo B-cell prolymphocytic leukemia with central nervous system involvement: description of an unreported complication. 61
18929411 2009
39
[Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia]. 61
19110524 2008
40
T-cell prolymphocytic leukemia: a report of two cases with review of literature. 61
23100959 2008
41
De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome. 61
27264708 2008
42
Quantitation of cytological parameters of malignant lymphocytes using computerized image analysis. 61
18665824 2008
43
Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia. 61
16997373 2007
44
IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). 61
16642047 2006
45
Allogeneic peripheral stem-cell transplantation with reduced-intensity conditioning regimen in refractory primary B-cell prolymphocytic leukemia: a long-term follow-up. 61
15880130 2005
46
Fibromodulin, an extracellular matrix protein: characterization of its unique gene and protein expression in B-cell chronic lymphocytic leukemia and mantle cell lymphoma. 61
15741214 2005
47
Prolymphocytic leukemia. 61
15869731 2005
48
[Clinicopathologic study of 369 B-cell non-Hodgkin lymphoma cases, with reference to the 2001 World Health Organization classification of lymphoid neoplasms]. 61
16091170 2005
49
Detection of CD5 in B-cell chronic lymphoproliferative diseases by flow cytometry: a strong expression in B-cell chronic lymphocytic leukemia. 61
16186976 2005
50
Successful treatment of B-cell prolymphocytic leukemia with monoclonal anti-CD20 antibody. 61
15060751 2004

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

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 ZAP70 MS4A1 IL2RA IGKC FCER2 CD5
2 external side of plasma membrane GO:0009897 9.1 MS4A1 IL2RA IGKC FCER2 CD5 CD19

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

# Name GO ID Score Top Affiliating Genes
1 B cell activation GO:0042113 9.46 ZAP70 MS4A1
2 negative regulation of fibroblast proliferation GO:0048147 9.43 TP53 MYC
3 chromosome organization GO:0051276 9.4 TP53 MYC
4 response to gamma radiation GO:0010332 9.37 TP53 MYC
5 Notch signaling pathway GO:0007219 9.33 MYC IL2RA FCER2
6 protein tetramerization GO:0051262 9.32 TP53 MS4A1
7 positive regulation of T cell differentiation GO:0045582 9.26 ZAP70 IL2RA
8 cytokine-mediated signaling pathway GO:0019221 9.26 TP53 MYC IL2RA FCER2
9 B cell receptor signaling pathway GO:0050853 8.92 MS4A1 IGKC CD38 CD19

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

# Name GO ID Score Top Affiliating Genes
1 core promoter sequence-specific DNA binding GO:0001046 8.62 TP53 MYC

Sources for B Cell Prolymphocytic Leukemia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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