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Hydroa vacciniforme-like lymphoma

MedGen UID:
317758
Concept ID:
C1708397
Neoplastic Process
Synonyms: Angiocentric cutaneous T-cell lymphoma of childhood; angiocentric cutaneous T-cell lymphoma of childhood; HV-Like LPD; hV-like lymphoma; HVLL; Hydroa Vacciniforme Lymphoproliferative Disorder; Hydroa vacciniforme-like cutaneous T-cell lymphoma; hydroa vacciniforme-like cutaneous T-cell lymphoma; hydroa vacciniforme-like lymphoma; Hydroa vacciniforme-like lymphoproliferative disorder; Hydroa Vacciniforme-Like Lymphoproliferative Disorder; hydroa vacciniforme-like lymphoproliferative disorder; Hydroa-like cutaneous T-cell lymphoma; hydroa-like cutaneous T-cell lymphoma
SNOMED CT: Hydroa vacciniforme-like lymphoma (450907007); Hydroa vacciniforme-like lymphoma (763719001); Hydroa vacciniforme-like lymphoproliferative disorder (789440003); Hydroa vacciniforme-like cutaneous T-cell lymphoma (1153397003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018224
Orphanet: ORPHA364039

Definition

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, hepatosplenomegaly and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHydroa vacciniforme-like lymphoma

Professional guidelines

PubMed

Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602

Recent clinical studies

Therapy

Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R, Alderete G, Grube P, Lome-Maldonado C, Bonzheim I, Fend F
Blood 2013 Oct 31;122(18):3101-10. Epub 2013 Aug 27 doi: 10.1182/blood-2013-05-502203. PMID: 23982171
Li Y, Chen XH, Tian XY, Li B, Li Z
Int J Surg Pathol 2013 Apr;21(2):161-8. Epub 2012 Jul 17 doi: 10.1177/1066896912453200. PMID: 22807582
Park S, Lee DY, Kim WS, Ko YH
Am J Dermatopathol 2010 Dec;32(8):832-6. doi: 10.1097/DAD.0b013e3181d68381. PMID: 20595887

Prognosis

López de Cáceres CVB, Rodrigues-Fernandes CI, Rendón Henao J, Morais TML, Soares CD, de Almeida OP, Fonseca FP, Delgado-Azañero W
J Oral Pathol Med 2021 Jul;50(6):530-539. Epub 2021 Jun 29 doi: 10.1111/jop.13203. PMID: 34097774
Yang YQ, Fan L, Wang L, Xu J, Zhang R, Ge Z, Li JY, Xu W
Int J Clin Exp Pathol 2014;7(9):6403-8. Epub 2014 Aug 15 PMID: 25337300Free PMC Article
Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R, Alderete G, Grube P, Lome-Maldonado C, Bonzheim I, Fend F
Blood 2013 Oct 31;122(18):3101-10. Epub 2013 Aug 27 doi: 10.1182/blood-2013-05-502203. PMID: 23982171
Wang M, Wang S, Yang QP, Liu YM, Gao LM, Sun H, Liu WP
Diagn Pathol 2013 May 1;8:72. doi: 10.1186/1746-1596-8-72. PMID: 23635342Free PMC Article
Li Y, Chen XH, Tian XY, Li B, Li Z
Int J Surg Pathol 2013 Apr;21(2):161-8. Epub 2012 Jul 17 doi: 10.1177/1066896912453200. PMID: 22807582

Clinical prediction guides

Ruiz R, Morante Z, Mantilla R, Mas L, Casanova L, Gomez HL
An Bras Dermatol 2017 Sep-Oct;92(5):649-654. doi: 10.1590/abd1806-4841.20176825. PMID: 29166501Free PMC Article
Magaña M, Massone C, Magaña P, Cerroni L
Am J Dermatopathol 2016 Jan;38(1):20-5. doi: 10.1097/DAD.0000000000000385. PMID: 26368647
Plaza JA, Sangueza M
Am J Dermatopathol 2015 Jan;37(1):20-5. doi: 10.1097/DAD.0000000000000158. PMID: 25162933
Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R, Alderete G, Grube P, Lome-Maldonado C, Bonzheim I, Fend F
Blood 2013 Oct 31;122(18):3101-10. Epub 2013 Aug 27 doi: 10.1182/blood-2013-05-502203. PMID: 23982171
Wang M, Wang S, Yang QP, Liu YM, Gao LM, Sun H, Liu WP
Diagn Pathol 2013 May 1;8:72. doi: 10.1186/1746-1596-8-72. PMID: 23635342Free PMC Article

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