Symptoma

Persistent Polyclonal B-cell Lymphocytosis

PPBL

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

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

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

  • Pathologist

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

    Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M. [books.google.com]

  • Splenomegaly

    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. [ncbi.nlm.nih.gov]

  • Massive Splenomegaly

    We present here one of the first detailed descriptions of the morphologic and immunohistochemical features of the spleen from a young female with PPBL who developed massive splenomegaly during 6-year follow up. [ncbi.nlm.nih.gov]

    Massive splenomegaly is rare among these patients. Our PPBL patient reported here manifested slowly progressive splenomegaly during six years of follow-up. [diagnosticpathology.biomedcentral.com]

    […] mutations in persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. ( 28679739 ) Tesson B....Sujobert P. 2017 3 B-cell signaling in persistent polyclonal B lymphocytosis (PPBL). ( 27126628 ) Voelxen N...Warnatz K 2016 4 Symptomatic Massive [malacards.org]

  • Long Arm

    FISH studies using a chromosome 3 long arm-specific probe provided evidence that all cases had both i(3)(q10) and 3. [ncbi.nlm.nih.gov]

  • Ulcer

    Research output : Chapter in Book/Report/Conference proceeding › Chapter (Book) › Research ABPI reporting and compression recommendations in global clinical practice guidelines on venous leg ulcer management: A scoping review Weller, C. [monash.edu]

  • Suggestibility

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

    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]

  • 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]

  • HLA-DR7

    Persistent polyclonal B-cell lymphocytosis with binucleate lymphocytes is a rare lymphoproliferative syndrome of uncertain cause that is strongly associated with HLA-DR7 positivity, cigarette smoking, and female sex. [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]

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]

Number and Phenotype in the Course of Pregnancy in a Woman with Persistent Polyclonal B-Cell Lymphocytosis: A Flow Cytometric Study. ( 26490519 ) Carulli G....Petrini M. 2015 6 Recurrence of persistent polyclonal B lymphocytosis (PPBL) after rituximab treatment [malacards.org]

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]

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-Lymphoma. [en.wikipedia.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]

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]

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]

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]

PPBL was first reported in 1982, it is a uncommon type of lymphocytosis of unknown etiology, features include female gender, cigarette smoking, polyclonal increase in immunoglobulin M (IgM), presence of circulating binucleated lymphocyte, as well as an [imagebank.hematology.org]

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]

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

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

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]