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persistent polyclonal B-cell lymphocytosis (PPBL) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

persistent polyclonal B-cell lymphocytosis

MONDO:0011672

Also known as:PPBLpersistent polyclonal B-cell lymphocytosis with binucleated lymphocytes

Overview

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare, generally benign, lymphoproliferative hematological disease characterized 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.

GeneticsAutosomal dominant

ResearchLimited published research (3 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include: Decreased circulating total IgM, Recurrent infections, Enlarged liver (hepatomegaly), and Increased total lymphocyte count and 1 more.

Organ System	Phenotype Count	Example Features
Blood and immune system	2	Recurrent infections, Enlarged spleen (splenomegaly)

Diagnosis

Biomarker and diagnostic research for persistent polyclonal B-cell lymphocytosis has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for persistent polyclonal B-cell lymphocytosis.

3 publications have been identified in PubMed for persistent polyclonal B-cell lymphocytosis. Research spans Diagnostic / Biomarker (33%), Case Report / Case Series (33%), and Basic Science / Preclinical (33%).

Recent Publications

Guarnera L (2026). [PMID: 40983034](https://pubmed.ncbi.nlm.nih.gov/40983034/). *Blood*. [Basic Science / Preclinical]
Rodgers M (2025). [PMID: 41205250](https://pubmed.ncbi.nlm.nih.gov/41205250/). *Journal of comparative pathology*. [Case Report / Case Series]
Laganà A (2025). [PMID: 39830795](https://pubmed.ncbi.nlm.nih.gov/39830795/). *Mediterranean journal of hematology and infectious diseases*. [Diagnostic / Biomarker]

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 6:06 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:606445

Orphanet

European rare disease database

Orphanet:300324

GARD

Genetic and Rare Diseases Info Center

GARD:17366

Questions for Your Care Team

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persistent polyclonal B-cell lymphocytosis

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Orphanet

GARD

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Questions for Your Care Team

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