Evaluating a splenectomy sample in cases like this may present a diagnostic challenge to pathologists. [ncbi.nlm.nih.gov]

We also present here a literature review on pathogenesis of PPBL. [thescholarship.ecu.edu]

RESULTS: PPBL presents in the majority of cases with persistent polyclonal B-cell lymphocytosis and affects primarily smoker women. [idibgi.org]

Physical examination was normal, especially no lymphadenopathy or hepatosplenomegaly were present. [ntvg.nl]

• Asymptomatic

  CONCLUSIONS: PPBL has an asymptomatic and stable evolution, although it frequently presents genetic abnormalities. It remains unknown whether it is a premalignant entity, similar to monoclonal gammopathies of unknown significance. [idibgi.org]

  The presence of characteristic binucleated lymphoid B-cells in asymptomatic family members and the description of familial PPBL cases suggest a genetic predisposition as a more likely possibility. Epidemiology PPBL was first reported in 1982. [atlasgeneticsoncology.org]

  Some patients report a varying degree of fatigue, consistent with a chronic fatigue syndrome,[1] or postviral fatigue as seen in EBV infections while others remain asymptomatic.[2] Genetics[edit] Genetically, PPBL has been associated with a few unusual [en.wikipedia.org]

  […] in bone marrow B cells, not proliferation of B cells in periphery or increased survival Clinical features Variable fatigue, consistent with chronic fatigue syndrome or post EBV fatigue 10% present with splenomegaly and lymphadenopathy but may also be asymptomatic [pathologyoutlines.com]

  The patients, smokers in 98% of cases, were either asymptomatic or had minor and nonspecific complaints, such as fatigue. Physical examination was usually within normal limits, except for a mild splenomegaly present in 10% of cases. [mafiadoc.com]

• Splenomegaly

  PPBL features an expansion of splenic marginal-zone B-lymphocytes, which infiltrate BM sinusoids and circulate in the blood with no evidence of clonality, even in cases with progressive splenomegaly. [ncbi.nlm.nih.gov]

  Reference Persistent Polyclonal B-cell Lymphocytosis With Splenomegaly: Histologic Description of 2 Cases. [humpath.com]

  Significant symptomatic splenomegaly requiring splenectomy is very unusual for this disorder; therefore there is a lack of descriptions of the morphologic features of the spleen in the literature. [thescholarship.ecu.edu]

• Erythema

  We report a case of PPBL with intermitting symptoms compatible with a chronic fatigue syndrome, recurrent erythema nodosum and multiforme. Serological findings suggested a chronic active Epstein-Barr virus (EBV) infection. [ncbi.nlm.nih.gov]

• Suggestibility

  An association with cigarette smoking was initially suggested. However PPBL was observed in non smokers patients. The morphology of binucleated lymphoid B-cells could suggest an association with viral infections, such as Epstein-Barr Virus. [atlasgeneticsoncology.org]

  Serological findings suggested a chronic active Epstein-Barr virus (EBV) infection. [ncbi.nlm.nih.gov]

• Seizure

  […] delay syndrome Lethal midline granuloma Lethal multiple congenital anomalies/dysmorphic syndrome Lethal multiple pterygium syndrome Lethal neonatal rigidity-multifocal seizure syndrome Lethal neonatal spasticity-epileptic encephalopathy syndrome Lethal [orpha.net]

[…] lymphocytic infiltrates (rather than plasma cells) found between glands, and destructive lymphoepithelial lesions in which intraepithelial lymphocytes express CD20 (B cells). [30] The morphologic examination is essential in selecting cases for further workup [emedicine.medscape.com]

General Approach to the Workup of Lymphocytosis A general approach to evaluating patients with lymphocytosis is shown in the Figure. [hematologyandoncology.net]

• Lymphocytosis

  Chronic B-cell lymphocytosis of the young woman : clinical, phenotypic, and molecular studies. Delage B, Darveau A, Jacques L, Huot A, Delage JM Blood,. 1992. Persistent polyclonal lymphocytosis of B lymphocytes. [atlasgeneticsoncology.org]

  […] polyclonal B-cell lymphocytosis is reported. [serval.unil.ch]

  B-cell lymphocytosis. ( 10502320 ) Callet-Bauchu E....Felman P. 1999 40 Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis. ( 9665185 ) Roy J....Delage R. 1998 41 All patients with persistent polyclonal B cell lymphocytosis present [malacards.org]

  Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare clinical condition, characterized by a persistent, generally moderate lymphocytosis, generally due to stimulation of central memory B-lymphocytes, and by a moderate increase of polyclonal IgM [ncbi.nlm.nih.gov]

Authored by international experts in the field, it covers a broad range of hematologic disorders -- both benign and malignant -- with information on the pathogenesis, clinical and pathologic diagnosis, and treatment for each. [books.google.com]

No definitively established treatment; recommended to avoid aggressive treatment Microscopic (histologic) description Bone marrow biopsies show lymphoid hyperplasia in a background of normal trilineage maturation Intrasinusoidal distribution of B cells [pathologyoutlines.com]

All 4 labs are meanwhile coordinating MRD investigations within study protocols for the treatment of different lymphatic and have developed quantitative PCR methods (Real-Time-PCR) for standardized MRD quantification in the setting of prospective clinical [european-mcl.net]

Morphologic features showing typical binucleated cells Prognosis After a median follow-up of 5.5 years without treatment, 45 PPBL patients are alive. Chronic B-cell lymphocytosis of the young woman : clinical, phenotypic, and molecular studies. [atlasgeneticsoncology.org]

Morphologic features showing typical binucleated cells Prognosis After a median follow-up of 5.5 years without treatment, 45 PPBL patients are alive. Chronic B-cell lymphocytosis of the young woman : clinical, phenotypic, and molecular studies. [atlasgeneticsoncology.org]

[citation needed] Prognosis[edit] In the followup of 111 patients, most remained stable and event free. However, two patients developed IgM gammopathy 2 lung cancer; one developed cervical cancer and three developed non-Hodgkin's lymphoma. [en.wikipedia.org]

Forkhead box protein P1 expression in mucosa-associated lymphoid tissue lymphomas predicts poor prognosis and transformation to diffuse large B-cell lymphoma. J Clin Oncol. 2006 Jun 1. 24(16):2490-7. [Medline]. Musshoff K. [emedicine.medscape.com]

NOTCH1 mutations in +12 chronic lymphocytic leukemia (CLL) confer an unfavorable prognosis, induce a distinctive transcriptional profiling and refine the intermediate prognosis of +12 CLL. Haematologica. 2012;97(3):437-441. 39. [hematologyandoncology.net]

Natural history of chronic lymphocytic leukemia: on the progression and progression and prognosis of early clinical stages. Nouv Rev Fr Hematol 1988 ;30: 359 - 361 19. Rawstron AC, Green MJ, Kuzmicki A, et al. [nejm.org]

Etiology The etiology of polyclonal B lymphocytosis with binucleated lymphocytes (PPBL) remains unknown. An association with cigarette smoking was initially suggested. However PPBL was observed in non smokers patients. [atlasgeneticsoncology.org]

This rare hematological disorder of unknown etiology is characterized by morphologically atypical lymphocytes, polyclonal immunoglobulin M production in association with smoking, female gender, and HLA-DR7 phenotype. [ncbi.nlm.nih.gov]

TERMINOLOGY Synonyms Reactive lymphocytosis Definitions Elevated blood lymphocyte count above age-defined normal range ETIOLOGY/PATHOGENESIS Etiology Lymphocytosis with reactive morphology Viral infections, such as EBV, CMV, or other Lymphocytosis with [basicmedicalkey.com]

Epidemiology PPBL was first reported in 1982. We have no epidemiological data on the incidence of PPBL. [atlasgeneticsoncology.org]

[…] stimulation possible Chronic NK-cell lymphocytosis Clonality assessment difficult Often associated with cutaneous disorders, vascular diseases, or malignancy Pathogenesis unclear; expansion in response to antigen stimulation possible CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Gastric MALT lymphoma: epidemiology and high adenocarcinoma risk in a nation-wide study. Eur J Cancer. 2008 Nov. 44(16):2470-6. [Medline]. Wöhrer S, Troch M, Streubel B, Zwerina J, Skrabs C, Formanek M, et al. [emedicine.medscape.com]

Whole blood cryopreservation in epidemiological studies. Cancer Epidemiol Biomarkers Prev 2002 ;11: 1496 - 1498 30. Giudicelli V, Chaume D, Lefranc MP. [nejm.org]

However, this type of data is clearly difficult to collect in epidemiological settings. [journals.plos.org]

The pathophysiology of PPBL is unknown, but its association with HLA DR 7 suggests a genetic predisposition. [ntvg.nl]

Terminology Also known as persistent polyclonal B lymphocytosis (PPBL), B cell expansion with NFKB and T cell anergy (BENTA) Sites Peripheral blood, bone marrow May also involve spleen and lymph node Pathophysiology Familial cases have germline heterozygous [pathologyoutlines.com]

The high Ig diversity with absence of stereotypes and the distinctive immunophenotype of PPBL are suggestive of a different pathophysiology than in mature B-cell malignancies. [nature.com]

A better description of mechanisms mediating B-cell clonal proliferation and survival will ultimately enhance our understanding of the pathophysiology of MBL and CLL and allow for the identification of new molecular targets. [nejm.org]

[…] ultimately will develop monoclonal B-cell lymphoma or B-cell leukemia.38,39 SECONDARY (REACTIVE) LYMPHOCYTOSIS Secondary lymphocytosis defines conditions associated with an increase in the absolute number of lymphocytes secondary to a physiologic or pathophysiologic [medtextfree.wordpress.com]

Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection. [books.google.com]

To prevent extensive work-up, knowledge of this disease entity is mandatory. [ntvg.nl]

& Brooks, D., Mar 2019, In : Journal of Cardiopulmonary Rehabilitation and Prevention. 39, 2, p. 85-90 6 p. [monash.edu]

We thank Christine Berg and Philip Prorok of the Division of Cancer Prevention, National Cancer Institute; the Screening Center investigators and staff of the PLCO Cancer Screening Trial; Tom Riley and the staff of Information Management Services; Barbara [nejm.org]

The early identification of lymphoma allows optimal long-term management and prevention of significant morbidity. [thedoctorsdoctor.com]