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ORPHA:364039

Hydroa vacciniforme-like lymphoma

medium confidenceDisorder

Also known as: Angiocentric cutaneous T-cell lymphoma of childhood · HVLL · Hydroa-like cutaneous T-cell lymphoma

Publications

228

63.1th percentile

Trials

1

Interventional, condition-specific

Researchers

1,014

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A very rare Epstein-Barr virus-associated lymphoproliferative disorder characterized by a chronic, recurrent, vesiculopapular rash, which subsequently ulcerates and scars, located mainly on sun-exposed areas and which is associated with systemic manifestations, such as fever, weight loss, asthenia, facial edema, arthralgia, lymphadenopathy, and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (6)

angiocentric cutaneous T-cell lymphoma of childhood · hV-like lymphoma · hydroa vacciniforme-like cutaneous T-cell lymphoma · hydroa vacciniforme-like lymphoma · hydroa vacciniforme-like lymphoproliferative disorder · hydroa-like cutaneous T-cell lymphoma

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.”

2/6 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

    228 matched papers (146 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 designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    1 matched on ClinicalTrials.gov (1 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)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

228

228 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=3967) is 59.

228 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).

146 in the last 10 years · medium confidence · 63.1th percentile (publications denominator)

Phrase hits: 228 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,014

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
    Jaffe ES13 papers · 2022

    Hematopathology Section, Laboratory of Pathology, National Cancer Institute, Bethesda, MD.

    Papers in Europe PMC
  2. 02
    Wang L13 papers · 2024

    Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China.

    Papers in Europe PMC
  3. 03
    Kimura H11 papers · 2022

    Department of Virology, Nagoya University Graduate School of Medicine, Nagoya.

    Papers in Europe PMC
  4. 04
    Barrionuevo C6 papers · 2022

    Department of Pathology, National Institute of Neoplastic Diseases, National University of San Marcos, 15038 Lima, Peru.

    Papers in Europe PMC
  5. 05
    Gru AA6 papers · 2023

    Department of Pathology, University of Colorado, Aurora, CO 80045, USA.

    Papers in Europe PMC
  6. 06
    Ko YH6 papers · 2021

    Department of Pathology, Samsung Medical Center, Sungkyunkwan University, Seoul, Korea.

    Papers in Europe PMC
  7. 07
    Li Y6 papers · 2025

    Department of Hematology, Xiangya Hospital of Central South University, Changsha, Hunan 410008, P.R. China.

    Papers in Europe PMC
  8. 08
    Quintanilla-Martinez L6 papers · 2022

    Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany.

    Papers in Europe PMC
  9. 09
    Cohen JI5 papers · 2022

    Medical Virology Section, Laboratory of Clinical Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.

    Papers in Europe PMC
  10. 10
    Wang T5 papers · 2022

    Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; 1 currently recruiting in our sample.

Data as of 11 September 2026

1 interventional trial — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 80.1th percentile).

medium confidence · 80.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

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.

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 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · 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).

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 Hydroa vacciniforme-like lymphoma — 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.

"Hydroa vacciniforme-like lymphoma" OR "Angiocentric cutaneous T-cell lymphoma of childhood" OR "Angiocentric cutaneous T-cell lymphoma of the childhood" OR "Hydroa-like cutaneous T-cell lymphoma" OR "hV-like lymphoma" OR "hydroa vacciniforme-like cutaneous T-cell lymphoma" OR "hydroa vacciniforme-like lymphoproliferative disorder"

Run this search on Europe PMC

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

"Hydroa vacciniforme-like lymphoma" OR "Angiocentric cutaneous T-cell lymphoma of childhood" OR "Angiocentric cutaneous T-cell lymphoma of the childhood" OR "Hydroa-like cutaneous T-cell lymphoma" OR "hV-like lymphoma" OR "hydroa vacciniforme-like cutaneous T-cell lymphoma" OR "hydroa vacciniforme-like lymphoproliferative disorder"

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: 1 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: HVLL

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 2026-07-27T14:46:42.342Z