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]

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

• Splenectomy

  Splenectomy was performed for symptomatic relief and suspicion of malignant process. [ncbi.nlm.nih.gov]

  Transient decreases in IgM levels seen post-splenectomy return to pre-splenectomy levels by 4 years( 14 ). This phenomenon would not explain the low IgM seen in the patient described here in, as the splenectomy was more than a decade prior. [ispub.com]

  This patient developed 2 years after splenectomy (13 years after PPBL diagnosis) a pulmonary diffuse large B cell lymphoma (DLBCL). [omicsonline.org]

• Pathologist

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

  Comprehensive in scope, it's a must-have resource for both residents and practicing pathologists alike. Authored by the chief architects of the WHO classification in neoplasms of hematopoietic and lymphoid tissue. [books.google.com]

• Multiple Congenital Anomalies

  Lethal Larsen-like syndrome Lethal left ventricular non-compaction-seizures-hypotonia-cataract-developmental delay syndrome Lethal midline granuloma Lethal multiple congenital anomalies/dysmorphic syndrome Lethal multiple pterygium syndrome Lethal neonatal [orpha.net]

• 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]

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

• 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]

  Mild splenomegaly is the most frequently reported physical finding, which is detected in about 10% of patients according to the largest case series [ 2 ]. Massive splenomegaly is rare among these patients. [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]

• 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]

  Suggestions for identifying the disorder are made. [ncbi.nlm.nih.gov]

[…] 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]

• Streptococcus Pneumoniae

  Immunoglobulin D/immunology ; Immunoglobulin G/immunology ; Immunoglobulin M/immunology ; Immunophenotyping ; Lymphocytosis/immunology/therapy ; Middle Aged ; Pneumococcal Infections/immunology/therapy ; Pneumococcal Vaccines/administration & dosage/immunology ; Streptococcus [orbi.ulg.ac.be]

• HLA-DR7

  Other notable findings in this case are lack of HLA-DR7 and strong positive CD5 markers in the polyclonal B-cell population. [mayoclinic.pure.elsevier.com]

  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]

  This recently identified distinct clinicopathologic entity is frequently associated with the presence of the HLA-DR7 antigen. It follows a benign course and has so far been reported only in women smokers. [serval.unil.ch]

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]

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]

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]

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]

& 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]