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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-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. See: This record Not genetically inherited (Orphanet)

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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVHydroa vacciniforme-like lymphoma

Disease
- Neoplasm
  - Neoplasm by Special Category
    - Childhood neoplasm
      - Childhood malignant neoplasm
        EBV-positive T-cell lymphoproliferative disorder of childhood
        Hydroa vacciniforme-like lymphoma

Professional guidelines

PubMed

Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.

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

See all (1)

Recent clinical studies

Etiology

Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases.

Liu Y, Ma C, Wang G, Wang L
J Am Acad Dermatol 2019 Aug;81(2):534-540. Epub 2019 Jan 14 doi: 10.1016/j.jaad.2019.01.011. PMID: 30654082

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

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

Paediatric cutaneous lymphomas: a review and comparison with adult counterparts.

Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K
J Eur Acad Dermatol Venereol 2015 Sep;29(9):1696-709. Epub 2015 Feb 25 doi: 10.1111/jdv.13044. PMID: 25715748

Cutaneous nonmycotic T- and natural killer/T-cell lymphomas: diagnostic challenges and dilemmas.

Chuang SS, Ko YH
J Am Acad Dermatol 2014 Apr;70(4):724-735. Epub 2014 Feb 9 doi: 10.1016/j.jaad.2013.11.047. PMID: 24521829

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.

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

See all (10)

Diagnosis

Oral manifestations of Hydroa vacciniforme-like lymphoproliferative disorder: a clinicopathological study of a Peruvian population.

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

Epstein-Barr virus and skin.

Sangueza-Acosta M, Sandoval-Romero E
An Bras Dermatol 2018 Nov/Dec;93(6):786-799. doi: 10.1590/abd1806-4841.20187021. PMID: 30484521 Free PMC Article

Hydroa vacciniforme-like lymphoma.

Qiao J, Fang H
QJM 2016 Oct;109(10):697-698. Epub 2016 Aug 9 doi: 10.1093/qjmed/hcw137. PMID: 27507019

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

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

Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature.

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: 23635342 Free PMC Article

See all (16)

Therapy

Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.

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

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.

Primary cutaneous hydroa vacciniforme-like lymphoma with indolent clinical course: report of two cases and review of literature.

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

Primary cutaneous Epstein-Barr virus-associated T-cell lymphoproliferative disorder-2 cases with unusual, prolonged clinical course.

Park S, Lee DY, Kim WS, Ko YH
Am J Dermatopathol 2010 Dec;32(8):832-6. doi: 10.1097/DAD.0b013e3181d68381. PMID: 20595887

See all (4)

Prognosis

Oral manifestations of Hydroa vacciniforme-like lymphoproliferative disorder: a clinicopathological study of a Peruvian population.

Paediatric cutaneous lymphomas: a review and comparison with adult counterparts.

Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K
J Eur Acad Dermatol Venereol 2015 Sep;29(9):1696-709. Epub 2015 Feb 25 doi: 10.1111/jdv.13044. PMID: 25715748

Systemic lymphoma arising from hydroa vacciniforme-like lymphoma: report of two cases with review of literature.

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: 25337300 Free PMC Article

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.

Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature.

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: 23635342 Free PMC Article

See all (13)

Clinical prediction guides

Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute.

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: 29166501 Free PMC Article

Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients.

Magaña M, Massone C, Magaña P, Cerroni L
Am J Dermatopathol 2016 Jan;38(1):20-5. doi: 10.1097/DAD.0000000000000385. PMID: 26368647

Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma.

Plaza JA, Sangueza M
Am J Dermatopathol 2015 Jan;37(1):20-5. doi: 10.1097/DAD.0000000000000158. PMID: 25162933

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.

Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature.

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: 23635342 Free PMC Article

See all (6)