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Persistent polyclonal B-cell lymphocytosis(PPBL)

MedGen UID:
341117
•Concept ID:
C1847973
•
Disease or Syndrome
Synonym: PPBL
SNOMED CT: Persistent polyclonal B-cell lymphocytosis (763864008); Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes (763864008); PPBL - persistent polyclonal B-cell lymphocytosis (763864008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0011672
OMIM®: 606445
Orphanet: ORPHA300324

Definition

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). [from OMIM]

Clinical features

From HPO
Hepatomegaly
MedGen UID:
42428
•Concept ID:
C0019209
•
Finding
Abnormally increased size of the liver.
See: Feature record | Search on this feature
Lymphocytosis
MedGen UID:
9834
•Concept ID:
C0024282
•
Disease or Syndrome
Increase in the number or proportion of lymphocytes in the blood.
See: Feature record | Search on this feature
Splenomegaly
MedGen UID:
52469
•Concept ID:
C0038002
•
Finding
Abnormal increased size of the spleen.
See: Feature record | Search on this feature
Decreased circulating total IgM
MedGen UID:
116095
•Concept ID:
C0239989
•
Finding
An abnormally decreased level of immunoglobulin M (IgM) in blood.
See: Feature record | Search on this feature
Recurrent infections
MedGen UID:
65998
•Concept ID:
C0239998
•
Finding
Increased susceptibility to infections.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPersistent polyclonal B-cell lymphocytosis
Follow this link to review classifications for Persistent polyclonal B-cell lymphocytosis in Orphanet.

Professional guidelines

PubMed

Cytogenetics in the management of clonal chromosomal abnormalities of undetermined significance and persistent polyclonal B-cell lymphocytosis: Guidelines from the Groupe Francophone de Cytogénétique Hématologique (GFCH).
Nadal N, Auger N, Bidet A, Nguyen-Khac F
Curr Res Transl Med 2023 Oct-Dec;71(4):103426. Epub 2023 Oct 26 doi: 10.1016/j.retram.2023.103426. PMID: 38016423

Recent clinical studies

Etiology

Persistent polyclonal binucleated B-cell lymphocytosis and MECOM gene amplification.
Cornet E, Mossafa H, Courel K, Lesesve JF, Troussard X
BMC Res Notes 2016 Mar 2;9:138. doi: 10.1186/s13104-015-1742-3. PMID: 26935937Free PMC Article
Persistent polyclonal B-cell lymphocytosis in chronic smokers: more than meets the eye.
Dasanu CA, Codreanu I
Conn Med 2012 Feb;76(2):69-72. PMID: 22670354
Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly.
Del Giudice I, Pileri SA, Rossi M, Sabattini E, Campidelli C, Starza ID, De Propris MS, Mancini F, Perrone MP, Gesuiti P, Armiento D, Quattrocchi L, Tafuri A, Amendola A, Mauro FR, Guarini A, Foà R
Br J Haematol 2009 Mar;144(5):726-31. Epub 2008 Dec 26 doi: 10.1111/j.1365-2141.2008.07551.x. PMID: 19133977
Familial persistent polyclonal B-cell lymphocytosis.
Himmelmann A, Rüegg R, Fehr J
Leuk Lymphoma 2001 Mar;41(1-2):157-60. doi: 10.3109/10428190109057965. PMID: 11342368
All patients with persistent polyclonal B cell lymphocytosis present Bcl-2/Ig gene rearrangements.
Delage R, Roy J, Jacques L, Darveau A
Leuk Lymphoma 1998 Nov;31(5-6):567-74. doi: 10.3109/10428199809057616. PMID: 9922047

Diagnosis

Cytogenetics in the management of clonal chromosomal abnormalities of undetermined significance and persistent polyclonal B-cell lymphocytosis: Guidelines from the Groupe Francophone de Cytogénétique Hématologique (GFCH).
Nadal N, Auger N, Bidet A, Nguyen-Khac F
Curr Res Transl Med 2023 Oct-Dec;71(4):103426. Epub 2023 Oct 26 doi: 10.1016/j.retram.2023.103426. PMID: 38016423
Successful Pregnancy and Persistent Polyclonal B Cell Lymphocytosis (PPBL): A Case Study of a Rare Co-Existence.
Dryllis G, Giannikos T, Konstantinou EA, Moustakas I, Christopoulos P, Pittaras T, Politou M, Valsami S
Am J Case Rep 2021 Dec 22;22:e933746. doi: 10.12659/AJCR.933746. PMID: 34936642Free PMC Article
Polyclonal B-cell lymphocytosis: Report of three cases.
Marcondes NA, Schneider L, Spindler BM, Fernandes FB, Faulhaber GAM
Cytometry B Clin Cytom 2018 Nov;94(6):953-955. Epub 2018 May 24 doi: 10.1002/cyto.b.21638. PMID: 29704326
Persistent polyclonal binucleated B-cell lymphocytosis and MECOM gene amplification.
Cornet E, Mossafa H, Courel K, Lesesve JF, Troussard X
BMC Res Notes 2016 Mar 2;9:138. doi: 10.1186/s13104-015-1742-3. PMID: 26935937Free PMC Article
Persistent polyclonal B-cell lymphocytosis.
Lesesve JF, Troussard X
Blood 2011 Dec 15;118(25):6485. doi: 10.1182/blood-2011-01-331082. PMID: 22279623

Therapy

SDHA gain-of-function engages inflammatory mitochondrial retrograde signaling via KEAP1-Nrf2.
Burgener AV, Bantug GR, Meyer BJ, Higgins R, Ghosh A, Bignucolo O, Ma EH, Loeliger J, Unterstab G, Geigges M, Steiner R, Enamorado M, Ivanek R, Hunziker D, Schmidt A, Müller-Durovic B, Grählert J, Epple R, Dimeloe S, Lötscher J, Sauder U, Ebnöther M, Burger B, Heijnen I, Martínez-Cano S, Cantoni N, Brücker R, Kahlert CR, Sancho D, Jones RG, Navarini A, Recher M, Hess C
Nat Immunol 2019 Oct;20(10):1311-1321. Epub 2019 Sep 16 doi: 10.1038/s41590-019-0482-2. PMID: 31527833
Persistent polyclonal B-cell lymphocytosis in chronic smokers: more than meets the eye.
Dasanu CA, Codreanu I
Conn Med 2012 Feb;76(2):69-72. PMID: 22670354
Selective defect of anti-pneumococcal IgG in a patient with persistent polyclonal B cell lymphocytosis.
Hafraoui K, Moutschen M, Smet J, Mascart F, Schaaf-Lafontaine N, Fillet G
Eur J Intern Med 2009 May;20(3):e62-5. Epub 2008 Oct 1 doi: 10.1016/j.ejim.2008.08.005. PMID: 19393481
Distinct chromosome 3 abnormalities in persistent polyclonal B-cell lymphocytosis.
Callet-Bauchu E, Gazzo S, Poncet C, Pagès J, Morel D, Alliot C, Coiffier B, Coeur P, Salles G, Felman P
Genes Chromosomes Cancer 1999 Nov;26(3):221-8. doi: 10.1002/(sici)1098-2264(199911)26:3<221::aid-gcc6>3.0.co;2-9. PMID: 10502320

Prognosis

Clinical course and features of persistent polyclonal B-cell lymphocytosis with BCL-6 amplification during pregnancy.
Galitzia A, Murru R, Caocci G, Barabino L, Azzena A, Licheri VM, Greco M, La Nasa G
Eur Rev Med Pharmacol Sci 2023 Apr;27(8):3514-3518. doi: 10.26355/eurrev_202304_32124. PMID: 37140301
Successful Pregnancy and Persistent Polyclonal B Cell Lymphocytosis (PPBL): A Case Study of a Rare Co-Existence.
Dryllis G, Giannikos T, Konstantinou EA, Moustakas I, Christopoulos P, Pittaras T, Politou M, Valsami S
Am J Case Rep 2021 Dec 22;22:e933746. doi: 10.12659/AJCR.933746. PMID: 34936642Free PMC Article
Persistent polyclonal binucleated B-cell lymphocytosis and MECOM gene amplification.
Cornet E, Mossafa H, Courel K, Lesesve JF, Troussard X
BMC Res Notes 2016 Mar 2;9:138. doi: 10.1186/s13104-015-1742-3. PMID: 26935937Free PMC Article
Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly.
Del Giudice I, Pileri SA, Rossi M, Sabattini E, Campidelli C, Starza ID, De Propris MS, Mancini F, Perrone MP, Gesuiti P, Armiento D, Quattrocchi L, Tafuri A, Amendola A, Mauro FR, Guarini A, Foà R
Br J Haematol 2009 Mar;144(5):726-31. Epub 2008 Dec 26 doi: 10.1111/j.1365-2141.2008.07551.x. PMID: 19133977
All patients with persistent polyclonal B cell lymphocytosis present Bcl-2/Ig gene rearrangements.
Delage R, Roy J, Jacques L, Darveau A
Leuk Lymphoma 1998 Nov;31(5-6):567-74. doi: 10.3109/10428199809057616. PMID: 9922047

Clinical prediction guides

B-cell signaling in persistent polyclonal B lymphocytosis (PPBL).
Voelxen N, Wehr C, Gutenberger S, Keller B, Erlacher M, Dominguez-Conde C, Bertele D, Emmerich F, Pantic M, Jennings S, Rakhmanov M, Foerster C, Martens UM, Platzbecker U, Peter HH, Fisch P, Boztug K, Eibel H, Salzer U, Warnatz K
Immunol Cell Biol 2016 Oct;94(9):830-837. Epub 2016 Apr 29 doi: 10.1038/icb.2016.46. PMID: 27126628
Persistent polyclonal binucleated B-cell lymphocytosis and MECOM gene amplification.
Cornet E, Mossafa H, Courel K, Lesesve JF, Troussard X
BMC Res Notes 2016 Mar 2;9:138. doi: 10.1186/s13104-015-1742-3. PMID: 26935937Free PMC Article
Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma.
Sun P, Juskevicius R
Diagn Pathol 2012 Aug 19;7:107. doi: 10.1186/1746-1596-7-107. PMID: 22901769Free PMC Article
Familial persistent polyclonal B-cell lymphocytosis.
Himmelmann A, Rüegg R, Fehr J
Leuk Lymphoma 2001 Mar;41(1-2):157-60. doi: 10.3109/10428190109057965. PMID: 11342368
Role of Epstein-Barr virus and soluble CD21 in persistent polyclonal B-cell lymphocytosis.
Larcher C, Fend F, Mitterer M, Prang N, Schwarzmann F, Huemer HP
Br J Haematol 1995 Jul;90(3):532-40. doi: 10.1111/j.1365-2141.1995.tb05580.x. PMID: 7646990

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