RARE DISEASERESEARCH ATLAS

ORPHA:300324

Persistent polyclonal B-cell lymphocytosis

medium confidenceDisorder

Also known as: PPBL · Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes

Publications

100

43.8th percentile

Trials

0

Interventional, condition-specific

Researchers

548

Distinct authors in sample

Gene link

Readiness

1/6

Stages with a signal

Clinical definition (Orphanet)

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 .

How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (2)

persistent polyclonal B-cell lymphocytosis · persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

1/6 stages with a signal

Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    100 matched papers (30 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialNot found

    No matched interventional trial under our ClinicalTrials.gov rules

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

100

100 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

100 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

30 in the last 10 years · medium confidence · 43.8th percentile (publications denominator)

Phrase hits: 100 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

548

Distinct author names in 100 sampled papers — named people below.

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Troussard X11 papers · 2021

    Laboratoire d'Hématologie Côte de Nacre, Université Caen Basse Normandie Caen, Registre Régional des Hémopathies Malignes de Basse Normandie, France.

    Papers in Europe PMC
  2. 02
    Mossafa H10 papers · 2014

    Département de génétique humaine, Laboratoire Pasteur-Cerba, Cergy-Pontoise, France.

    Papers in Europe PMC
  3. 03
    Lesesve JF9 papers · 2021

    Service d'Hématologie Biologique, CHU Brabois , Nancy , France.

    Papers in Europe PMC
  4. 04
    Delage R7 papers · 2014

    Centre d'Hématologie et d'Immunologie Clinique, Hôpital du St-Sacrement, Laval University, Quebec, Ste-Foy, Canada.

    Papers in Europe PMC
  5. 05
    Darveau A6 papers · 2014

    Département de Biochimie, de Microbiologie et de Bio-Informatique, Pavillon Alexandre-Vachon, 1045 avenue de la Médecine, Bureau 3428, Université Laval, Québec, QC, Canada G1V 0A6.

    Papers in Europe PMC
  6. 06
    Cornet E5 papers · 2014

    Laboratoire d'hématologie, CHU Côte de Nacre, Caen, France.

    Papers in Europe PMC
  7. 07
    Huemer HP4 papers · 2005
    Papers in Europe PMC
  8. 08
    Larcher C4 papers · 2005

    Institute for Hygiene, University of Innsbruck, Austria.

    Papers in Europe PMC
  9. 09
    Mitterer M4 papers · 2005

    Department of Haematology, General Hospital, Bozen, Italy.

    Papers in Europe PMC
  10. 10
    Callet-Bauchu E3 papers · 2017

    Laboratoire Central d'Hématologie, Centre Hospitalier Lyon Sud, Pierre-Bénite, France.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name. 1 observational study did — shown below because natural-history and cohort work can be an important step toward a trial.

Data as of 27 July 2026

No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.

medium confidence · 36.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.

Observational and natural-history studies

1 observational study matches this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

None of the matched observational studies is currently listed as recruiting.

Open the complete matched search on ClinicalTrials.gov

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Persistent polyclonal B-cell lymphocytosis" OR "Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Persistent Polyclonal B-Cell Lymphocytosis

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Persistent polyclonal B-cell lymphocytosis" OR "Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes"

Study-type breakdown: 0 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: PPBL

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T12:39:25.307Z