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Last update 08 May 2025

Persistent Polyclonal B-Cell Lymphocytosis

Last update 08 May 2025

Basic Info

Synonyms

PERSISTENT POLYCLONAL B-CELL LYMPHOCYTOSIS, PPBL, PPBL - persistent polyclonal B-cell lymphocytosis

+ [7]

Introduction

A rare generally benign lymphoproliferative haematological disease characterised by chronic, stable, persistent, polyclonal lymphocytosis of memory B-cell origin, the presence of binucleated lymphocytes in the peripheral blood, and a polyclonal increase in serum immunoglobulin M (IgM). Patients are most frequently asymptomatic or may present with mild splenomegaly.

Drugs associated with Persistent Polyclonal B-Cell Lymphocytosis

Clevudine

Target

HBV pol

Mechanism

HBV pol inhibitors

Active Org.

Guangxi Huibaoyuan Pharmaceutical Technology Co., Ltd. [+1]

Originator Org.

Bukwang Pharmaceutical Co., Ltd.

Active Indication

Hepatitis B [+1]

Inactive Indication

Complete atrioventricular block [+2]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

South Korea

First Approval Date13 Jun 2001

Clinical Trials associated with Persistent Polyclonal B-Cell Lymphocytosis

NCT03296046

/ CompletedNot ApplicableIIT

Epidemiologic Study of Persistent Polyclonal B-cell Lymphocytosis (PPBL) - Observatory 1990-2012

The main purpose of this study is the description of all French cases of PPBL and the establishment of minimal criteria for the pathology diagnosis and its follow-up.
The secondary objective is the immunologic and genetic characterization of lymphoid cells causing the disease.

Start Date13 Nov 2012

Sponsor / Collaborator

Centre Hospitalier Regional de Nancy

NCT00672867

/ CompletedPhase 3

A Open Randomized Clinical Trial Comparing the Efficacy and Safety of Clevudine 30mg Versus Adefovir 10mg in Patients With LC-B

The purpose of this study is to compare the safety and antiviral activity of Clevudine Versus Adefovir dipivoxil in patients with LC-B.

Start Date01 Dec 2007

Sponsor / Collaborator

Bukwang Pharmaceutical Co., Ltd.

100 Clinical Results associated with Persistent Polyclonal B-Cell Lymphocytosis

100 Translational Medicine associated with Persistent Polyclonal B-Cell Lymphocytosis

0 Patents (Medical) associated with Persistent Polyclonal B-Cell Lymphocytosis

407

Literatures (Medical) associated with Persistent Polyclonal B-Cell Lymphocytosis

01 Jul 2025·Clinical Immunology

Allogeneic hematopoietic stem cell transplantation in a patient with combined immunodeficiency caused by IRF4 mutation

Article

Author: Wang, Hongsheng ; Qian, Xiaowen ; Wang, Xiaochuan ; Zhai, Xiaowen ; Sun, Jinqiao ; Jiang, Wenjin ; Wang, Ping

Heterozygous mutations in the interferon regulatory factor 4 (IRF4) can lead to combined immunodeficiency in humans. We report a pediatric case involving a male patient who presented with recurrent pneumonia, chronic diarrhoea, abdominal pain, and a skin rash. Laboratory evaluation revealed hypoglobulinemia and persistent B-cell lymphopenia. Genetic analysis confirmed that the combined immunodeficiency was caused by an IRF4 mutation. Given the failure of conventional therapies, the patient successfully underwent allogeneic hematopoietic stem cell transplantation (HSCT) from an unrelated donor using a reduced-toxicity conditioning regimen. Post-transplant follow-up demonstrated successful immune reconstitution and complete resolution of gastrointestinal symptoms. This case provides clinical evidence supporting HSCT as a feasible strategy to reconstitute the immune system and resolve enteropathy in patients with IRF4 mutations.

01 Feb 2025·European Journal of Haematology

Immunophenotyping for the Assessment of Asymptomatic Lymphocytosis: A Retrospective Analysis and National Survey

Article

Author: Butler, Tom ; Assefa, Esubalew ; Johnstone, Peter ; Freeman, Tanya ; Farren, Timothy ; Hibbs, Stephen P. ; Araf, Shamzah

ABSTRACTAsymptomatic lymphocytosis poses a common challenge in haematology. Immunophenotyping can establish whether a clonal population is present, but it is expensive and the benefit of diagnosing asymptomatic patients is unproven. This study aimed to establish data to guide the use of immunophenotyping. We analysed the proportion of lymphocytosis in full blood count (FBC) samples across a five‐year period within a large UK National Health Service (NHS) trust. Persistent lymphocytosis was present in 0.18% (437/242678) of repeat community samples. Of samples sent for immunophenotyping, 743/784 (95%) with a lymphocyte count > 10 × 109/L had a clonal population, compared to 223/1696 (14%) with a lymphocyte count < 5 × 109/L. We followed up a longitudinal cohort of asymptomatic patients with clonal lymphocytosis to determine how many required treatment. The minority (11/46) of patients needed treatment within 9 years of follow‐up. Of patients needing treatment, 10/11 (91%) had a presenting lymphocyte count > 10 × 109/L. In all cases, treatment was initiated when the patient became symptomatic. We propose a lymphocyte count threshold of > 10 × 109/L for referral and immunophenotyping in patients with asymptomatic lymphocytosis. This approach aims to balance safety and cost‐effectiveness and reflects uncertainty in the value of diagnosis for asymptomatic patients.

01 Jan 2025·Journal of Dairy Science

Estimation of genetic parameters and genome-wide association study for enzootic bovine leukosis resistance in Canadian Holstein cattle

Article

Author: van Staaveren, Nienke ; Oliveira, Gerson A ; Schenkel, Flavio S ; Lynch, Colin ; Kelton, David F ; Baes, Christine F ; Houlahan, Kerry ; Miglior, Filippo ; Bongers, Renee ; Rojas de Oliveira, Hinayah ; Rochus, Christina M

Bovine leukemia virus (BLV) is the causative agent of enzootic bovine leukosis (hereafter referred to as leukosis), frequently observed in North American dairy herds. Infection with BLV can lead to persistent lymphocytosis and tumors and is associated with decreased production, immunity. and fertility. With no available treatment or vaccine, reducing the prevalence of leukosis through management and culling has not yet been successful. Genetic selection could contribute to permanent improvement in dairy cattle resistance to leukosis. This study aimed to examine the prevalence and impact of leukosis in Canada and to assess the potential for including leukosis resistance in Canadian national genetic evaluations by characterizing the genetic architecture of leukosis resistance using pedigree and genomic information. A total of 117,349 milk ELISA test records on 96,779 Holstein cows from 950 Canadian herds taken between 2007 and 2021 were provided by Lactanet Canada (Guelph, ON, Canada). Each cow was classified as test-positive for leukosis or test-negative for leukosis. Leukosis was present in ∼77% of herds tested; within those herds, an average of 39% of cows tested were test-positive for leukosis. Heritabilities of 0.10 (SE = 0.001) and 0.07 (SE <0.001) were estimated for leukosis resistance using a linear animal model and BLUP or single-step GBLUP methodology, respectively. Breeding value correlations were estimated between leukosis resistance and economically important and phenotypically relevant traits. Most correlations between leukosis resistance and traits already included in Canadian genetic evaluations were favorable, with the exception of SCS. The candidate genes for leukosis resistance identified using a genome-wide association study were on chromosome 23, with some being part of the major histocompatibility complex. This study showed that genetic evaluation for leukosis resistance is possible, and could be considered for inclusion in Canadian national selection indices.

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Persistent Polyclonal B-Cell Lymphocytosis

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