PPBL
MCID: PRS108
MIFTS: 36

Persistent Polyclonal B-Cell Lymphocytosis (PPBL)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Persistent Polyclonal B-Cell Lymphocytosis

MalaCards integrated aliases for Persistent Polyclonal B-Cell Lymphocytosis:

Name: Persistent Polyclonal B-Cell Lymphocytosis 57 58 72 29 70
Ppbl 57 58 72
Persistent Polyclonal B-Cell Lymphocytosis with Binucleated Lymphocytes 58
Lymphocytosis, B-Cell, Polyclonal, Persistent 39

Characteristics:

Orphanet epidemiological data:

58
persistent polyclonal b-cell lymphocytosis
Inheritance: Multigenic/multifactorial;

OMIM®:

57 (Updated 20-May-2021)
Inheritance:
autosomal dominant

Miscellaneous:
adult-onset
increased prevalence among women
increased prevalence among smokers


HPO:

31
persistent polyclonal b-cell lymphocytosis:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

OMIM® 57 606445
MeSH 44 D008218
ICD10 via Orphanet 33 D72.8
UMLS via Orphanet 71 C1847973
Orphanet 58 ORPHA300324
MedGen 41 C1847973
UMLS 70 C1847973

Summaries for Persistent Polyclonal B-Cell Lymphocytosis

UniProtKB/Swiss-Prot : 72 Persistent polyclonal B-cell lymphocytosis: An autosomal dominant condition characterized by onset in infancy of splenomegaly and polyclonal expansion of B cells, resulting in peripheral lymphocytosis. Affected individuals also show mild immune dysfunction, including some defective antibody responses and T-cell anergy. There may be a predisposition to the development of B-cell malignancy.

MalaCards based summary : Persistent Polyclonal B-Cell Lymphocytosis, also known as ppbl, is related to b-cell expansion with nfkb and t-cell anergy and b-cell lymphoma. An important gene associated with Persistent Polyclonal B-Cell Lymphocytosis is CARD11 (Caspase Recruitment Domain Family Member 11), and among its related pathways/superpathways are T cell receptor signaling pathway and Bacterial infections in CF airways. Affiliated tissues include b cells, bone marrow and eye, and related phenotypes are splenomegaly and hepatomegaly

OMIM® : 57 Persistent polyclonal B-cell lymphocytosis (PPBL) is characterized by chronic, stable, persistent, and polyclonal lymphocytosis, the presence of binucleated lymphocytes in the peripheral blood, and a polyclonal increase in serum IgM. It is significantly associated with cigarette smoking (summary by Cornet et al., 2009). (606445) (Updated 20-May-2021)

Wikipedia : 73 Persistent polyclonal B-cell lymphocytosis (PPBL) is an anomaly of the human immune system characterized... more...

Related Diseases for Persistent Polyclonal B-Cell Lymphocytosis

Diseases related to Persistent Polyclonal B-Cell Lymphocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 53, show less)
# Related Disease Score Top Affiliating Genes
1 b-cell expansion with nfkb and t-cell anergy 32.2 FCER2 CARD11
2 b-cell lymphoma 30.0 FCER2 FAS CARD11
3 burkitt lymphoma 29.5 IL4 FCER2 FAS
4 leukemia, chronic lymphocytic 29.5 IL4 FCER2 FAS
5 lymphoproliferative syndrome 10.6
6 splenomegaly 10.6
7 lung cancer susceptibility 3 10.2
8 lymphoplasmacytic lymphoma 10.2
9 marginal zone b-cell lymphoma 10.2
10 splenic marginal zone lymphoma 10.2
11 follicular lymphoma 10.2
12 lymphoma 10.2
13 erythema nodosum 10.2
14 tetanus 10.2
15 gastroenteritis 10.2
16 acute kidney failure 10.2
17 chronic fatigue syndrome 10.2
18 chromosomal triplication 10.2
19 diffuse large b-cell lymphoma 9.9
20 monoclonal gammopathy of uncertain significance 9.9
21 cerebral aneurysms 9.9
22 hypersensitivity reaction type iv disease 9.8 IL4 FAS
23 silicosis 9.8 IL4 FAS
24 pneumoconiosis 9.8 IL4 FAS
25 adult t-cell leukemia 9.8 IL4 FAS
26 trypanosomiasis 9.8 IL4 FAS
27 sezary's disease 9.8 IL4 CARD11
28 toxic oil syndrome 9.8 IL4 FCER2
29 intrinsic asthma 9.8 IL4 FCER2
30 autoimmune hepatitis 9.8 IL4 FAS
31 lymphopenia 9.8 IL4 FAS
32 chronic conjunctivitis 9.8 IL4 FCER2
33 relapsing-remitting multiple sclerosis 9.8 IL4 FAS
34 onchocerciasis 9.8 IL4 FCER2
35 viral hepatitis 9.8 IL4 FAS
36 cutaneous leishmaniasis 9.8 IL4 FCER2
37 omenn syndrome 9.8 IL4 CARD11
38 food allergy 9.8 IL4 FCER2
39 hashimoto thyroiditis 9.8 IL4 FAS
40 bullous pemphigoid 9.8 IL4 FCER2
41 human immunodeficiency virus infectious disease 9.7 IL4 FAS
42 cutaneous t cell lymphoma 9.7 IL4 FAS
43 urticaria 9.7 IL4 FCER2
44 rhinitis 9.7 IL4 FCER2
45 allergic disease 9.7 IL4 FCER2
46 graves' disease 9.6 IL4 FAS
47 allergic rhinitis 9.6 IL4 FCER2
48 viral infectious disease 9.6 IL4 FAS
49 common variable immunodeficiency 9.6 IL4 FAS CARD11
50 asthma 9.5 IL4 FCER2 FAS
51 dermatitis 9.5 IL4 FCER2 CARD11
52 behcet syndrome 9.5 IL4 FAS
53 malaria 9.4 IL4 FCER2 FAS

Graphical network of the top 20 diseases related to Persistent Polyclonal B-Cell Lymphocytosis:



Diseases related to Persistent Polyclonal B-Cell Lymphocytosis

Symptoms & Phenotypes for Persistent Polyclonal B-Cell Lymphocytosis

Human phenotypes related to Persistent Polyclonal B-Cell Lymphocytosis:

31 (showing 5, show less)
# Description HPO Frequency HPO Source Accession
1 splenomegaly 31 HP:0001744
2 hepatomegaly 31 HP:0002240
3 recurrent infections 31 HP:0002719
4 decreased circulating total igm 31 HP:0002850
5 lymphocytosis 31 HP:0100827

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Immunology:
b-cell lymphocytosis, b-cell, polyclonal
increased igm levels, polyclonal
binucleated lymphocytes

Clinical features from OMIM®:

606445 (Updated 20-May-2021)

Drugs & Therapeutics for Persistent Polyclonal B-Cell Lymphocytosis

Interventional clinical trials:

(showing 1, show less)
# Name Status NCT ID Phase Drugs
1 Epidemiologic Study of Persistent Polyclonal B-cell Lymphocytosis (PPBL) - Observatory 1990-2012 Completed NCT03296046

Search NIH Clinical Center for Persistent Polyclonal B-Cell Lymphocytosis

Genetic Tests for Persistent Polyclonal B-Cell Lymphocytosis

Genetic tests related to Persistent Polyclonal B-Cell Lymphocytosis:

# Genetic test Affiliating Genes
1 Persistent Polyclonal B-Cell Lymphocytosis 29

Anatomical Context for Persistent Polyclonal B-Cell Lymphocytosis

MalaCards organs/tissues related to Persistent Polyclonal B-Cell Lymphocytosis:

40
B Cells, Bone Marrow, Eye, Spleen, Kidney, Lung, Bone

Publications for Persistent Polyclonal B-Cell Lymphocytosis

Articles related to Persistent Polyclonal B-Cell Lymphocytosis:

(showing 87, show less)
# Title Authors PMID Year
1
Morphologic features of binucleated lymphocytes to assess the diagnosis of persistent B-cell polyclonal lymphocytosis or other mature B-cell neoplasms. 61 57
24033110 2014
2
Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. 61 57
18668130 2009
3
Persistent polyclonal B-cell lymphocytosis: further evidence for a genetic disorder associated with B-cell abnormalities. 57 61
11552996 2001
4
Persistent polyclonal B lymphocytosis with binucleated lymphocytes: a study of 25 cases. Groupe Français d'Hématologie Cellulaire. 57 61
10086784 1999
5
Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis. 57 61
9665185 1998
6
Persistent polyclonal lymphocytosis of B lymphocytes. 57
6979709 1982
7
Persistent Polyclonal B-cell Lymphocytosis with Binucleated Lymphocytes (PPBL). 61
33687875 2021
8
Eight-year follow up of a case of persistent polyclonal B cell lymphocytosis: Immunophenotypic findings pre- and postsplenectomy. 61
33270977 2020
9
Abnormal characteristic "round bottom flask" shape volume-based scattergram as a trigger to suspect persistent polyclonal B-cell lymphocytosis. 61
33068629 2020
10
Myocardial Dysfunction in Cirrhotic Cardiomyopathy is Associated with Alterations of Phospholamban Phosphorylation and IL-6 Levels. 61
33220932 2020
11
PromA Plasmids Are Instrumental in the Dissemination of Linuron Catabolic Genes Between Different Genera. 61
32132980 2020
12
SDHA gain-of-function engages inflammatory mitochondrial retrograde signaling via KEAP1-Nrf2. 61
31527833 2019
13
Polyclonal B-cell lymphocytosis: Report of three cases. 61
29704326 2018
14
Pure PBL, Hybrid PBL and Lecturing: which one is more effective in developing cognitive skills of undergraduate students in pediatric nursing course? 61
30097035 2018
15
Persistent polyclonal B-cell lymphocytosis: two nuclei and sometimes more. 61
29271126 2018
16
Modulation of periodontitis progression using pH-responsive nanosphere encapsulating metronidazole or N-phenacylthialzolium bromide. 61
28795395 2018
17
Absence of driver mutations in persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. 61
28679739 2017
18
Hairy B-Cell Lymphoproliferative Disorder and its Differential Diagnosis: a Case with Long-Term Follow-Up. 61
28894563 2017
19
B-cell signaling in persistent polyclonal B lymphocytosis (PPBL). 61
27126628 2016
20
Persistent polyclonal binucleated B-cell lymphocytosis and MECOM gene amplification. 61
26935937 2016
21
Recurrence of persistent polyclonal B lymphocytosis (PPBL) after rituximab treatment. 61
25638669 2015
22
Symptomatic Massive Splenomegaly in Persistent Polyclonal B-cell Lymphocytosis Requiring Splenectomy. 61
26693179 2015
23
Persistent polyclonal b-cell lymphocytosis: a diagnostic challenge. 61
23190164 2015
24
Modifications in B-Lymphocyte Number and Phenotype in the Course of Pregnancy in a Woman with Persistent Polyclonal B-Cell Lymphocytosis: A Flow Cytometric Study. 61
26490519 2015
25
Persistent polyclonal B-cell lymphocytosis: extensively proliferated CD27+IgM+IgD+ memory B cells with a distinctive immunophenotype. 61
24549258 2014
26
Persistent Polyclonal B Cell Lymphocytosis B Cells Can Be Activated through CD40-CD154 Interaction. 61
25580126 2014
27
[Morphologic features of the binucleated lymphocytes for the assesment of persistent B-cell polyclonal lymphocytosis]. 61
24342786 2013
28
[SAGA complex subunit Spt20 involves in the calcineurin-mediated Cl- homeostasis in Schizosaccharomyces pombe]. 61
24400488 2013
29
Persistent polyclonal B-cell lymphocytosis with splenomegaly: histologic description of 2 cases. 61
23715167 2013
30
Smoking-induced monoclonal B-lymphocytosis in two female smokers: what are the odds? 61
23923254 2013
31
Comparison of three problem-based learning conditions (real patients, digital and paper) with lecture-based learning in a dermatology course: a prospective randomized study from China. 61
23009254 2013
32
Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma. 61
22901769 2012
33
Persistent polyclonal B-cell lymphocytosis in chronic smokers: more than meets the eye. 61
22670354 2012
34
Persistent polyclonal B-cell lymphocytosis. 61
22279623 2011
35
IgM interference in determination of antiphospholipid antibodies and erythrocyte sedimentation rate in persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. 61
21435190 2011
36
EDTA-dependent lymphoagglutination in persistent polyclonal B-cell lymphocytosis. 61
21770917 2011
37
[Persistent polyclonal B-cell lymphocytosis: study of 35 cases]. 61
21396665 2011
38
[Persistent polyclonal B-cell lymphocytosis: benign disease or prelymphoma?]. 61
21371723 2011
39
[Persistent polyclonal B-cell lymphocytosis]. 61
21586183 2011
40
Selective defect of anti-pneumococcal IgG in a patient with persistent polyclonal B cell lymphocytosis. 61
19393481 2009
41
Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly. 61
19133977 2009
42
Expression profiling of persistent polyclonal B-cell lymphocytosis suggests constitutive expression of the AP-1 transcription complex and downregulation of Fas-apoptotic and TGFbeta signalling pathways. 61
18754033 2009
43
Combined automated cell and flow cytometric analysis enables recognition of persistent polyclonal B-cell lymphocytosis (PPBL), a study of 25 patients. 61
18587574 2008
44
Polyclonal B-cell lymphocytosis with binucleated lymphocytes (PPBL). 61
21127753 2008
45
High incidence of ancestral HLA haplotype 8.1 and monoclonal incomplete DH-JH immunoglobulin heavy chain gene rearrangement in persistent polyclonal B-cell lymphocytosis. 61
18180922 2008
46
[Polyclonal B-cell lymphocytosis with hairy cell appearance: hairy B-cell lymphoproliferative disorder]. 61
17867302 2007
47
[Asymptomatic 32 year old female smoker with persistent polyclonal lymphocytosis]. 61
17287965 2007
48
Persistent polyclonal B-cell lymphocytosis: a case report. 61
16988824 2006
49
Functional T-cell anergy in a case of persistent polyclonal B-cell lymphocytosis. 61
15979703 2005
50
Oral feeding with pig peripheral lymphocytes decreases the xenogeneic delayed type hypersensitivity reaction in galactosyltransferase knockout mice. 61
16298587 2005
51
Intravascular bone marrow accumulation in persistent polyclonal lymphocytosis: a misleading feature for B-cell neoplasm. 61
15143340 2004
52
Chromosomal instability and ATR amplification gene in patients with persistent and polyclonal B-cell lymphocytosis (PPBL). 61
15359640 2004
53
[Binucleated lymphocyte lymphocytosis]. 61
12915355 2003
54
[Persistent polyclonal B-cell lymphocytosis]. 61
12748901 2003
55
Dysfunction of the Fas apoptotic signaling pathway in persistent polyclonal B-cell lymphocytosis. 61
12604424 2003
56
Analysis of expressed V(H) genes in persistent polyclonal B cell lymphocytosis reveals absence of selection in CD27+IgM+IgD+ memory B cells. 61
12516560 2002
57
Dicentric chromosome 3 associated with binucleated lymphocytes in atypical B-cell chronic lymphoproliferative disorder. 61
12685827 2002
58
[Persistent polyclonal B-cell lymphocytosis]. 61
12050042 2002
59
Persistent polyclonal B lymphocytosis: an expansion of cells showing IgVH gene mutations and phenotypic features of normal lymphocytes from the CD27+ marginal zone B-cell compartment. 61
11849229 2002
60
Persistent polyclonal B-cell lymphocytosis. 61
12017755 2002
61
Persistent polyclonal B-cell lymphocytosis in a middle-aged, smoking woman with typical morphologic and genetic hallmarks. 61
11532639 2001
62
Persistent polyclonal B-cell lymphocytosis is an expansion of functional IgD(+)CD27(+) memory B cells. 61
11529864 2001
63
Lack of CD40-dependent B-cell proliferation in B lymphocytes isolated from patients with persistent polyclonal B-cell lymphocytosis. 61
11380461 2001
64
Familial persistent polyclonal B-cell lymphocytosis. 61
11342368 2001
65
No BCL-2 protein over expression but BCL-2/IgH rearrangements in B cells of patients with persistent polyclonal B-cell lymphocytosis. 61
11920254 2001
66
Persistent polyclonal B lymphocytosis: morphological, immunological, cytogenetic and molecular analysis of an Italian case. 61
10996207 2000
67
Persistent polyclonal B-cell lymphocytosis--an important differential diagnosis of B-cell chronic lymphocytic leukemia. 61
10901613 2000
68
Isochromosome +i(3)(q10) in a new case of persistent polyclonal B-cell lymphocytosis (PPBL) 61
10863981 2000
69
[Polyclonal B lymphocytosis and hyper-IgM: immunodeficiency and/or benign lymphoid proliferation associated with tobacco?]. 61
10763184 2000
70
Distinct chromosome 3 abnormalities in persistent polyclonal B-cell lymphocytosis. 61
10502320 1999
71
Bilobulated circulating lymphocytes in persistent polyclonal B-cell lymphocytosis. 61
10457412 1999
72
CD5+ persistent polyclonal B-cell lymphocytosis in a male. 61
10342588 1999
73
All patients with persistent polyclonal B cell lymphocytosis present Bcl-2/Ig gene rearrangements. 61
9922047 1998
74
[Persistent polyclonal B-cell lymphocytosis. A Western pattern versus and Oriental model?]. 61
9741221 1998
75
Persistent polyclonal B lymphocytosis with multiple bcl-2/IgH rearrangements: a benign disorder. 61
9592988 1998
76
[Cellular immune reactivity in xenogenic "human-anti-pig" transplant combination]. 61
9746979 1998
77
Distribution of the cytogenetic abnormality +i(3)(q10) in persistent polyclonal B-cell lymphocytosis: a FICTION study in three cases. 61
9401061 1997
78
Multiple bcl-2/Ig gene rearrangements in persistent polyclonal B-cell lymphocytosis. 61
9207405 1997
79
Persistent polyclonal B-cell lymphocytosis in identical twins. 61
9029012 1997
80
Epstein-Barr virus-associated persistent polyclonal B-cell lymphocytosis with a distinct 69-base pair deletion in the LMP1 oncogene. 61
9031611 1997
81
[Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes in a male patient]. 61
9161582 1997
82
Pathogenesis of chronic Epstein-Barr virus infection: detection of a virus strain with a high rate of lytic replication. 61
8982037 1996
83
Role of Epstein-Barr virus and soluble CD21 in persistent polyclonal B-cell lymphocytosis. 61
7646990 1995
84
Chronic active Epstein-Barr virus disease in a case of persistent polyclonal B-cell lymphocytosis. 61
7646989 1995
85
Persistent polyclonal B-cell lymphocytosis. 61
7934138 1994
86
[Persistent B-cell polyclonal lymphocytosis: a benign lymphoproliferative syndrome]. 61
1947954 1991
87
Persistent polyclonal B lymphocytosis with Epstein-Barr virus antibodies and subsequent malignant pulmonary blastoma. 61
1851502 1991

Variations for Persistent Polyclonal B-Cell Lymphocytosis

Expression for Persistent Polyclonal B-Cell Lymphocytosis

Search GEO for disease gene expression data for Persistent Polyclonal B-Cell Lymphocytosis.

Pathways for Persistent Polyclonal B-Cell Lymphocytosis

Pathways related to Persistent Polyclonal B-Cell Lymphocytosis according to GeneCards Suite gene sharing:

(showing 7, show less)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.67 IL4 FAS CARD11
2
Show member pathways
11.62 IL4 FAS
3 11.43 IL4 FCER2
4 11.32 IL4 FCER2
5 11.31 IL4 FAS CARD11
6 11.06 IL4 FCER2
7 10.85 IL4 FCER2

GO Terms for Persistent Polyclonal B-Cell Lymphocytosis

Biological processes related to Persistent Polyclonal B-Cell Lymphocytosis according to GeneCards Suite gene sharing:

(showing 5, show less)
# Name GO ID Score Top Affiliating Genes
1 B cell differentiation GO:0030183 9.32 IL4 CARD11
2 positive regulation of T cell proliferation GO:0042102 9.26 IL4 CARD11
3 T cell activation GO:0042110 9.16 IL4 CARD11
4 positive regulation of B cell proliferation GO:0030890 8.96 IL4 CARD11
5 extrinsic apoptotic signaling pathway in absence of ligand GO:0097192 8.62 IL4 FAS

Sources for Persistent Polyclonal B-Cell Lymphocytosis

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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