ORPHA:364039
Hydroa vacciniforme-like lymphoma
Also known as: Angiocentric cutaneous T-cell lymphoma of childhood · HVLL · Hydroa-like cutaneous T-cell lymphoma
Publications
228
73.4th 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.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0018224
- UMLS:C1708397
- NCIT:C45327
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.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
228 matched papers (146 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- 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)
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?
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=5449) is 41.
228 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).
146 in the last 10 years · medium confidence · 73.4th percentile (publications denominator)
Phrase hits: 228 · MeSH hits: 0
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.
- 01Jaffe ES13 papers · 2022
Hematopathology Section, Laboratory of Pathology, National Cancer Institute, Bethesda, MD.
Papers in Europe PMC - 02Wang L13 papers · 2024
Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China.
Papers in Europe PMC - 03Kimura H11 papers · 2022
Department of Virology, Nagoya University Graduate School of Medicine, Nagoya.
Papers in Europe PMC - 04Barrionuevo C6 papers · 2022
Department of Pathology, National Institute of Neoplastic Diseases, National University of San Marcos, 15038 Lima, Peru.
Papers in Europe PMC - 05Gru AA6 papers · 2023
Department of Pathology, University of Colorado, Aurora, CO 80045, USA.
Papers in Europe PMC - 06Ko YH6 papers · 2021
Department of Pathology, Samsung Medical Center, Sungkyunkwan University, Seoul, Korea.
Papers in Europe PMC - 07Li Y6 papers · 2025
Department of Hematology, Xiangya Hospital of Central South University, Changsha, Hunan 410008, P.R. China.
Papers in Europe PMC - 08Quintanilla-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 - 09Cohen JI5 papers · 2022
Medical Virology Section, Laboratory of Clinical Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Papers in Europe PMC - 10Wang 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 27 July 2026
1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).
medium confidence · 76.8th 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.
- NCT05475925·RECRUITING·A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas
Conditions: LGLL - Large Granular Lymphocytic Leukemia · Primary Cutaneous Gamma-Delta T-Cell Lymphoma · Primary Cutaneous CD8+ Aggressive Epidermotropic T-Cell Lymphoma · Hepatosplenic T-cell Lymphoma·Matched via name phrase
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
- NCT05978141·RECRUITING·A Registry for People With T-cell Lymphoma
Conditions: T-cell Lymphoma · NK-Cell Lymphoma · T-cell Prolymphocytic Leukemia · T-cell Large Granular Lymphocytic Leukemia·Matched via name phrase
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 2026-07-26This 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"
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