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ORPHA:86871

T-cell prolymphocytic leukemia

low confidenceDisorder

Also known as: T-PLL · T-cell chronic lymphocytic leukemia

Publications

2,683

Trials

27

Interventional, condition-specific

Researchers

1,564

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell , involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with , generalized lymphadenopathy, high leukocyte count with normal serum immunoglobulins, anemia, and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (10)

CLL, T-cell · T cell CLL · T cell chronic lymphocytic leukaemia · T cell chronic lymphocytic leukemia · T cell prolymphocytic leukaemia · T cell prolymphocytic leukemia · T prolymphocytic leukaemia · T prolymphocytic leukemia · T-cell CLL · T-cell chronic lymphocytic leukaemia

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.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    2,683 matched papers (1,297 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPresent

    1 FDA · 1 EMA designations (1 FDA orphan-indication approval) — e.g. tinostamustine hydrochloride Source

  6. Interventional trialPresent

    27 matched on ClinicalTrials.gov (7 recruiting in sample)

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)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

2

Designations · 1 with FDA orphan-indication approval

  • FDA tinostamustine hydrochlorideT-Cell Prolymphocytic Leukemia · 2019-03-15 · Not FDA Approved for Orphan Indication
  • EMA tinostamustineTreatment of T-cell prolymphocytic leukaemia · 27/07/2020 · PositiveEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

17

Drugs / clinical candidates · MONDO_0019468

CTD chemicals (MyDisease.info)

1 associated chemical · 38 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Bortezomib · therapeutic

Pathways: ErbB signaling pathway; Cytokine-cytokine receptor interaction; Chemokine signaling pathway; Jak-STAT signaling pathway; Th1 and Th2 cell differentiation; Th17 cell differentiation; Prolactin signaling pathway; AGE-RAGE signaling pathway in diabetic complications

MyDisease.info · MONDO:0019468

Literature

Is anyone studying this?

2,683

2,683 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

2,683 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

1,297 in the last 10 years · low confidence

Phrase hits: 2,680 · MeSH hits: 3

Open Europe PMC search

Who's working on it?

1,564

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Herling M15 papers · 2026

    Department I of Internal Medicine, Center for Integrated Oncology (CIO), Aachen-Bonn-Cologne-Duesseldorf, Excellence Cluster for Cellular Stress Response and Aging-Associated Diseases (CECAD), Center for Molecular Medicine Cologne (CMMC), University of Cologne (UoC), Cologne, Germany.

    Papers in Europe PMC
  2. 02
    Braun T10 papers · 2026

    Department I of Internal Medicine, Center for Integrated Oncology (CIO), Aachen-Bonn-Cologne-Duesseldorf, Excellence Cluster for Cellular Stress Response and Aging-Associated Diseases (CECAD), Center for Molecular Medicine Cologne (CMMC), University of Cologne (UoC), Cologne, Germany.

    Papers in Europe PMC
  3. 03
    Schrader A7 papers · 2026

    Department I of Internal Medicine, Center for Integrated Oncology (CIO) Köln-Bonn, Excellence Cluster for Cellular Stress Response and Aging-Associated Diseases (CECAD), Center for Molecular Medicine Cologne (CMMC), University of Cologne, Cologne, Germany.

    Papers in Europe PMC
  4. 04
    Wahnschaffe L6 papers · 2026

    Department I of Internal Medicine, Center for Integrated Oncology Aachen-Bonn-Cologne-Düsseldorf, University Hospital Cologne, Cologne, Germany.

    Papers in Europe PMC
  5. 05
    Ferrajoli A5 papers · 2026

    MD Anderson Cancer Center, Houston, United States of America

    Papers in Europe PMC
  6. 06
    Aittokallio T4 papers · 2025

    Institute for Molecular Medicine Finland (FIMM), HiLIFE, University of Helsinki, Helsinki, Finland.

    Papers in Europe PMC
  7. 07
    Dechow A4 papers · 2025

    Department I of Internal Medicine, Center for Integrated Oncology Aachen-Bonn-Cologne-Düsseldorf, University Hospital Cologne, Cologne, Germany.

    Papers in Europe PMC
  8. 08
    Dürig J4 papers · 2022

    Department of Hematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.

    Papers in Europe PMC
  9. 09
    Franitza M4 papers · 2026

    Cologne Center for Genomics, Center for Molecular Medicine Cologne, University of Cologne, Cologne, Germany.

    Papers in Europe PMC
  10. 10
    Hallek M4 papers · 2026

    Department I of Internal Medicine, Center for Integrated Oncology Aachen-Bonn-Cologne-Düsseldorf, University Hospital Cologne, Cologne, Germany.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

27

interventional trials for this specific condition

27 interventional trials matched this specific condition name; 7 currently recruiting in our sample. 68 trials are registered for prolymphocytic leukemia, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

27 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 95.7th percentile).

low confidence · 95.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

27 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: prolymphocytic leukemia

68

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

2 observational studies match 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.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 8 · after dedupe 8 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 8 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (8)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for T-cell prolymphocytic leukemia — check Associations / patient organisations on that page.

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.

"T-cell prolymphocytic leukemia" OR "T-PLL" OR "T-cell chronic lymphocytic leukemia" OR "CLL, T-cell" OR "T cell CLL" OR "T cell chronic lymphocytic leukaemia" OR "T cell chronic lymphocytic leukemia" OR "T cell prolymphocytic leukaemia" OR "T cell prolymphocytic leukemia" OR "T prolymphocytic leukaemia" OR "T prolymphocytic leukemia" OR "T-cell CLL" OR "T-cell chronic lymphocytic leukaemia"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Leukemia, Prolymphocytic, T-Cell

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

"T-cell prolymphocytic leukemia" OR "T-PLL" OR "T-cell chronic lymphocytic leukemia" OR "CLL, T-cell" OR "T cell CLL" OR "T cell chronic lymphocytic leukaemia" OR "T cell chronic lymphocytic leukemia" OR "T cell prolymphocytic leukaemia" OR "T cell prolymphocytic leukemia" OR "T prolymphocytic leukaemia" OR "T prolymphocytic leukemia" OR "T-cell CLL" OR "T-cell chronic lymphocytic leukaemia" OR "Leukemia, Prolymphocytic, T-Cell"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 27 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"prolymphocytic leukemia"

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

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (2683) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T03:14:56.284Z