Hydroa vacciniforme-like lymphoma: Difference between revisions

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== Overview ==
== Overview ==
Hydroa vacciniforme-like lymphoma is a [[EBV]]-positive [[peripheral T-cell lymphoma]] ([[Non-Hodgkin's lymphoma]]), that affects primarily pediatric patients and has a bad prognosis.<ref name="pmid11893040">{{cite journal| author=Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F et al.| title=Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. | journal=Appl Immunohistochem Mol Morphol | year= 2002 | volume= 10 | issue= 1 | pages= 7-14 | pmid=11893040 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11893040  }} </ref> It is associated with [[photosensitivity]] and insect bites.<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref> Clinically, it manifests with vacciniform vesicles, necrotic ulcers and scars.<ref>{{cite web|url=http://www.dermatol-sinica.com/web/data/20101230112942.pdf|title=Hydroa vacciniforme-like lymphoma: a case report and literature review}}</ref>
Hydroa vacciniforme-like lymphoma (HVLL) is an [[EBV]]-positive [[peripheral T-cell lymphoma]] ([[non-Hodgkin's lymphoma]]), that affects primarily pediatric patients and has a bad prognosis.<ref name="pmid11893040">{{cite journal| author=Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F et al.| title=Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. | journal=Appl Immunohistochem Mol Morphol | year= 2002 | volume= 10 | issue= 1 | pages= 7-14 | pmid=11893040 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11893040  }} </ref> HVLL is associated with [[photosensitivity]] and insect bites.<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref> Clinically, HVLL manifests with vacciniform vesicles, necrotic ulcers, and scars.<ref>{{cite web|url=http://www.dermatol-sinica.com/web/data/20101230112942.pdf|title=Hydroa vacciniforme-like lymphoma: a case report and literature review}}</ref> Some cases of Hydroa vacciniforme-like lymphoma express [[NK-cell]] surface receptors.<ref name="pmid20497358">{{cite journal| author=Xu Z, Lian S| title=Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children. | journal=Pediatr Dermatol | year= 2010 | volume= 27 | issue= 5 | pages= 463-9 | pmid=20497358 | doi=10.1111/j.1525-1470.2010.01094.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20497358  }} </ref>


==Historical Perspective==
==Historical Perspective==
Hydroa vacciniforme-like lymphoma reports date back from 1986,<ref name="pmid3490832">{{cite journal| author=Oono T, Arata J, Masuda T, Ohtsuki Y| title=Coexistence of hydroa vacciniforme and malignant lymphoma. | journal=Arch Dermatol | year= 1986 | volume= 122 | issue= 11 | pages= 1306-9 | pmid=3490832 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3490832  }} </ref> although only since 2008, the WHO recognizes the HVLL as a separate entity from the Hydroa vacciniforme and classified it under <i>"EBV positive T-cell lymphoproliferative disorders of childhood".<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref></i>
HVLL reports date back to 1986;<ref name="pmid3490832">{{cite journal| author=Oono T, Arata J, Masuda T, Ohtsuki Y| title=Coexistence of hydroa vacciniforme and malignant lymphoma. | journal=Arch Dermatol | year= 1986 | volume= 122 | issue= 11 | pages= 1306-9 | pmid=3490832 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3490832  }} </ref> however, it is not until 2008 that the WHO recognized HVLL as a separate entity from Hydroa vacciniforme and classified it under <i>"EBV positive T-cell lymphoproliferative disorders of childhood".<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref></i>


==Classification==
==Pathophysiology==


==Pathophysiology==
===Histopathology===
Histopathologic examination demonstrates lymphocytic infiltrate in the skin, that are characterized by:<ref name="pmid23541598">{{cite journal| author=Sangueza M, Plaza JA| title=Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases. | journal=J Am Acad Dermatol | year= 2013 | volume= 69 | issue= 1 | pages= 112-9 | pmid=23541598 | doi=10.1016/j.jaad.2013.01.037 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23541598  }} </ref>
*Angiotropism
*Angiocentricity


==Causes==
===Immunophenotype===
*[[CD8]] positive<ref name="pmid23541598">{{cite journal| author=Sangueza M, Plaza JA| title=Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases. | journal=J Am Acad Dermatol | year= 2013 | volume= 69 | issue= 1 | pages= 112-9 | pmid=23541598 | doi=10.1016/j.jaad.2013.01.037 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23541598  }} </ref>
*Some cases are [[CD4]] positive<ref name="pmid17910721">{{cite journal| author=Wu YH, Chen HC, Hsiao PF, Tu MI, Lin YC, Wang TY| title=Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child. | journal=Int J Dermatol | year= 2007 | volume= 46 | issue= 10 | pages= 1081-6 | pmid=17910721 | doi=10.1111/j.1365-4632.2007.03102.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?.dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17910721  }} </ref>
* Always express [[granzyme B]] and [[T-cell intracellular antigen 1]] ([[TIA-1]]) independently of the cell type derivation<ref>{{cite web|url=http://www.bloodjournal.org/content/122/18/3101.long?sso-checked=true#ref-13|title=Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma}}</ref>
*CD45RO or [[CD56]] positive<ref name="pmid20497358">{{cite journal| author=Xu Z, Lian S| title=Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children. | journal=Pediatr Dermatol | year= 2010 | volume= 27 | issue= 5 | pages= 463-9 | pmid=20497358 | doi=10.1111/j.1525-1470.2010.01094.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20497358}}</ref>
*Monoclonal-type rearrangement of the T-cell receptor gamma<ref name="pmid11893040">{{cite journal| author=Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F et al.| title=Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. | journal=Appl Immunohistochem Mol Morphol | year= 2002 | volume= 10 | issue= 1 | pages= 7-14 | pmid=11893040 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11893040  }} </ref>
*[[CD30]] positive<ref name="pmid9580256">{{cite journal| author=Magaña M, Sangüeza P, Gil-Beristain J, Sánchez-Sosa S, Salgado A, Ramón G et al.| title=Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): a distinctive type of cutaneous T-cell lymphoma. | journal=J Am Acad Dermatol | year= 1998 | volume= 38 | issue= 4 | pages= 574-9 | pmid=9580256 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9580256  }} </ref>


==Differential Diagnosis==
==Differential Diagnosis==
*[[Extranodal T-/NK-cell lymphoma, nasal type]]  
*[[Extranodal T-/NK-cell lymphoma, nasal type]]  
*[[Mycosis fungoides]]  
*[[Mycosis fungoides]]  
*[[Precursor T-cell lymphoblastic lymphoma]]
*Precursor T-cell lymphoblastic lymphoma
*[[Peripheral T-cell lymphoma, not otherwise characterized]]
*[[Peripheral T-cell lymphoma, not otherwise characterized]]
 
*Primary cutaneous anaplastic large cell lymphoma
*[[Primary cutaneous anaplastic large cell lymphoma]]
 
*[[Subcutaneous panniculitis-like T-cell lymphoma]]
*[[Subcutaneous panniculitis-like T-cell lymphoma]]
*[[Lupus]]<ref name="pmid25488018">{{cite journal| author=Kim TH, Lee JH, Kim YC, Lee SE| title=Hydroa vacciniforme-like lymphoma misdiagnosed as cutaneous lupus erythematosus. | journal=J Cutan Pathol | year= 2014 | volume=  | issue=  | pages=  | pmid=25488018 | doi=10.1111/cup.12425 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25488018  }} </ref>
*[[Herpes simplex]]<ref name="pmid23782639">{{cite journal| author=Wang T, Wang L, Xiong L, Han M, Liu W, Li G| title=A rare case of hydroa vacciniforme-like cutaneous T-cell lymphoma presenting mimicking herpes simplex in an adult. | journal=Am J Dermatopathol | year= 2013 | volume= 35 | issue= 5 | pages= 617-8 | pmid=23782639 | doi=10.1097/DAD.0b013e318277053c | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23782639  }} </ref><ref name="pmid11893040">{{cite journal| author=Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F et al.| title=Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. | journal=Appl Immunohistochem Mol Morphol | year= 2002 | volume= 10 | issue= 1 | pages= 7-14 | pmid=11893040 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11893040  }} </ref>


== Epidemiology and Demographics ==
== Epidemiology and Demographics ==
===Age===
HVLL occurs mainly in the pediatric population.<ref name="pmid23982171">{{cite journal| author=Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R et al.| title=Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma. | journal=Blood | year= 2013 | volume= 122 | issue= 18 | pages= 3101-10 | pmid=23982171 | doi=10.1182/blood-2013-05-502203 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23982171  }} </ref> Although rare, it may present in adult population.<ref name="pmid24966953">{{cite journal| author=Wang RC, Chang ST, Hsieh YC, Huang WT, Hsu JD, Tseng CE et al.| title=Spectrum of Epstein-Barr virus-associated T-cell lymphoproliferative disorder in adolescents and young adults in Taiwan. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 5 | pages= 2430-7 | pmid=24966953 | doi= | pmc=PMC4069894 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24966953  }} </ref>


Hydroa vacciniforme-like lymphoma occurs mainly in the pediatric population from South America, Central America and Asia.<ref name="pmid23982171">{{cite journal| author=Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R et al.| title=Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma. | journal=Blood | year= 2013 | volume= 122 | issue= 18 | pages= 3101-10 | pmid=23982171 | doi=10.1182/blood-2013-05-502203 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23982171 }} </ref> Although rare, it may present in adult population.<ref name="pmid24966953">{{cite journal| author=Wang RC, Chang ST, Hsieh YC, Huang WT, Hsu JD, Tseng CE et al.| title=Spectrum of Epstein-Barr virus-associated T-cell lymphoproliferative disorder in adolescents and young adults in Taiwan. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 5 | pages= 2430-7 | pmid=24966953 | doi= | pmc=PMC4069894 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24966953 }} </ref>
===Gender===
In one study, there was difference in incidence when comparing men and women.<ref>{{cite web|url=http://journals.lww.com/appliedimmunohist/Abstract/2002/03000/Hydroa_Like_Cutaneous_T_Cell_Lymphoma__A.2.aspx|title=Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru}}</ref>
 
===Developing Countries===
HVLL occurs in South America, Central America, and Asia.<ref name="pmid23982171">{{cite journal| author=Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R et al.| title=Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma. | journal=Blood | year= 2013 | volume= 122 | issue= 18 | pages= 3101-10 | pmid=23982171 | doi=10.1182/blood-2013-05-502203 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23982171  }} </ref>


== Risk Factors ==
== Risk Factors ==
*[[Immunosupression]]


== Natural History, Complications, and Prognosis==
== Natural History, Complications, and Prognosis==
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=== History and Symptoms ===
=== History and Symptoms ===
* Skin lesions  may or may not appear in sunlight exposed areas:<ref name="pmid17976208">{{cite journal| author=Doeden K, Molina-Kirsch H, Perez E, Warnke R, Sundram U| title=Hydroa-like lymphoma with CD56 expression. | journal=J Cutan Pathol | year= 2008 | volume= 35 | issue= 5 | pages= 488-94 | pmid=17976208 | doi=10.1111/j.1600-0560.2007.00836.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17976208  }} </ref>
** [[Edema]]
** [[Blisters]]
** [[Ulcers]]
** Crusts
** Scars
* Typical [[B symptoms]]<ref name="pmid25072366">{{cite journal| author=Boddu D, George R, Nair S, Bindra M, G Mathew L| title=Hydroa Vacciniforme-Like Lymphoma: A Case Report From India. | journal=J Pediatr Hematol Oncol | year= 2014 | volume=  | issue=  | pages=  | pmid=25072366 | doi=10.1097/MPH.0000000000000221 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25072366  }} </ref>
* Periorbital swelling<ref name="pmid25162933">{{cite journal| author=Plaza JA, Sangueza M| title=Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma. | journal=Am J Dermatopathol | year= 2015 | volume= 37 | issue= 1 | pages= 20-5 | pmid=25162933 | doi=10.1097/DAD.0000000000000158 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25162933  }} </ref>
====Physical Examination====
*[[Lymphadenopathy]]
*[[Hepatosplenomegaly]]<ref>{{cite web|url=http://www.bloodjournal.org/content/122/18/3101?sso-checked=true|title=Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma}}</ref>
===Exploratory Findings===
*[[EBV]] [[DNA]] in peripheral blood
*[[EBV]] [[RNA]] in histological cuts of skin lesions with in situ hybridization
*Tumor cells positive for [[EBV]] [[RNA]]<ref name="pmid20497358">{{cite journal| author=Xu Z, Lian S| title=Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children. | journal=Pediatr Dermatol | year= 2010 | volume= 27 | issue= 5 | pages= 463-9 | pmid=20497358 | doi=10.1111/j.1525-1470.2010.01094.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20497358  }} </ref>


[[Edema]], [[blisters]], [[ulcers]], crusts and scars are evidenced in face and extremities of patients with Hydroa vacciniforme-like lymphomas. The mentioned lesions  may or may not appear in sunlight exposed areas.<ref name="pmid17976208">{{cite journal| author=Doeden K, Molina-Kirsch H, Perez E, Warnke R, Sundram U| title=Hydroa-like lymphoma with CD56 expression. | journal=J Cutan Pathol | year= 2008 | volume= 35 | issue= 5 | pages= 488-94 | pmid=17976208 | doi=10.1111/j.1600-0560.2007.00836.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17976208  }} </ref> Patients may present with typical [[B symptoms]],<ref name="pmid25072366">{{cite journal| author=Boddu D, George R, Nair S, Bindra M, G Mathew L| title=Hydroa Vacciniforme-Like Lymphoma: A Case Report From India. | journal=J Pediatr Hematol Oncol | year= 2014 | volume=  | issue=  | pages=  | pmid=25072366 | doi=10.1097/MPH.0000000000000221 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25072366  }} </ref> which in some cases, are preceded by periorbtal swelling.<ref name="pmid25162933">{{cite journal| author=Plaza JA, Sangueza M| title=Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma. | journal=Am J Dermatopathol | year= 2015 | volume= 37 | issue= 1 | pages= 20-5 | pmid=25162933 | doi=10.1097/DAD.0000000000000158 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25162933  }} </ref>
===Laboratory Findings===
*IgM positive for [[EBV]]


==Treatment==
==Treatment==
*No significant beneficial results have been achieved with [[chemotherapy]] and [[radiotherapy]].<ref name="Thalidomide">{{cite web|url=http://www.readcube.com/articles/10.1002%2Fajh.23843?r3_referer=wol&tracking_action=preview_click&show_checkout=1&purchase_referrer=onlinelibrary.wiley.com|title=Thalidomide for the treatment of hydroa vacciniforme-like lymphoma:Report of four pediatric cases from Peru}}</ref><ref>{{cite web|url=http://journals.lww.com/appliedimmunohist/Abstract/2002/03000/Hydroa_Like_Cutaneous_T_Cell_Lymphoma__A.2.aspx|title=Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru}}</ref>
*[[Thalidomide]] has been recently used for the treatment of HVLL.<ref name="Thalidomide">{{cite web|url=http://www.readcube.com/articles/10.1002%2Fajh.23843?r3_referer=wol&tracking_action=preview_click&show_checkout=1&purchase_referrer=onlinelibrary.wiley.com|title=Thalidomide for the treatment of hydroa vacciniforme-like lymphoma:Report of four pediatric cases from Peru}}</ref>
*Skin eruptions can be treated with intramuscular interferon alfa.<ref>{{cite web|url=http://onlinelibrary.wiley.com/doi/10.1111/j.1525-1470.2010.01094.x/abstract|title=Epstein–Barr Virus-Associated Hydroa Vacciniforme-like Cutaneous Lymphoma in Seven Chinese Children}}</ref>
*[[Glucocorticoids]] can successfully relieve symptoms associated with HVLL.<ref name="pmid19651001">{{cite journal| author=Li HY, Wang HL, Gao TZ, Zhuo ZH, Li DM, Li HX| title=[Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review]. | journal=Zhongguo Dang Dai Er Ke Za Zhi | year= 2009 | volume= 11 | issue= 7 | pages= 596-8 | pmid=19651001 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19651001  }} </ref>


==References==
==References==

Latest revision as of 22:55, 3 March 2015

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alberto Plate [2]

Synonyms and keywords: Hydroa-like CTCL, HVLL

Overview

Hydroa vacciniforme-like lymphoma (HVLL) is an EBV-positive peripheral T-cell lymphoma (non-Hodgkin's lymphoma), that affects primarily pediatric patients and has a bad prognosis.[1] HVLL is associated with photosensitivity and insect bites.[2] Clinically, HVLL manifests with vacciniform vesicles, necrotic ulcers, and scars.[3] Some cases of Hydroa vacciniforme-like lymphoma express NK-cell surface receptors.[4]

Historical Perspective

HVLL reports date back to 1986;[5] however, it is not until 2008 that the WHO recognized HVLL as a separate entity from Hydroa vacciniforme and classified it under "EBV positive T-cell lymphoproliferative disorders of childhood".[2]

Pathophysiology

Histopathology

Histopathologic examination demonstrates lymphocytic infiltrate in the skin, that are characterized by:[6]

  • Angiotropism
  • Angiocentricity

Immunophenotype

Differential Diagnosis

Epidemiology and Demographics

Age

HVLL occurs mainly in the pediatric population.[12] Although rare, it may present in adult population.[13]

Gender

In one study, there was difference in incidence when comparing men and women.[14]

Developing Countries

HVLL occurs in South America, Central America, and Asia.[12]

Risk Factors

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Exploratory Findings

  • EBV DNA in peripheral blood
  • EBV RNA in histological cuts of skin lesions with in situ hybridization
  • Tumor cells positive for EBV RNA[4]

Laboratory Findings

  • IgM positive for EBV

Treatment

References

  1. ↑ 1.0 1.1 1.2 Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F; et al. (2002). "Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru". Appl Immunohistochem Mol Morphol. 10 (1): 7–14. PMID 11893040.
  2. ↑ 2.0 2.1 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
  3. ↑ "Hydroa vacciniforme-like lymphoma: a case report and literature review" (PDF).
  4. ↑ 4.0 4.1 4.2 Xu Z, Lian S (2010). "Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children". Pediatr Dermatol. 27 (5): 463–9. doi:10.1111/j.1525-1470.2010.01094.x. PMID 20497358.
  5. ↑ Oono T, Arata J, Masuda T, Ohtsuki Y (1986). "Coexistence of hydroa vacciniforme and malignant lymphoma". Arch Dermatol. 122 (11): 1306–9. PMID 3490832.
  6. ↑ 6.0 6.1 Sangueza M, Plaza JA (2013). "Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases". J Am Acad Dermatol. 69 (1): 112–9. doi:10.1016/j.jaad.2013.01.037. PMID 23541598.
  7. ↑ Wu YH, Chen HC, Hsiao PF, Tu MI, Lin YC, Wang TY (2007). "Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child". Int J Dermatol. 46 (10): 1081–6. doi:10.1111/j.1365-4632.2007.03102.x. PMID 17910721.
  8. ↑ "Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma".
  9. ↑ Magaña M, Sangüeza P, Gil-Beristain J, Sánchez-Sosa S, Salgado A, Ramón G; et al. (1998). "Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): a distinctive type of cutaneous T-cell lymphoma". J Am Acad Dermatol. 38 (4): 574–9. PMID 9580256.
  10. ↑ Kim TH, Lee JH, Kim YC, Lee SE (2014). "Hydroa vacciniforme-like lymphoma misdiagnosed as cutaneous lupus erythematosus". J Cutan Pathol. doi:10.1111/cup.12425. PMID 25488018.
  11. ↑ Wang T, Wang L, Xiong L, Han M, Liu W, Li G (2013). "A rare case of hydroa vacciniforme-like cutaneous T-cell lymphoma presenting mimicking herpes simplex in an adult". Am J Dermatopathol. 35 (5): 617–8. doi:10.1097/DAD.0b013e318277053c. PMID 23782639.
  12. ↑ 12.0 12.1 Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R; et al. (2013). "Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma". Blood. 122 (18): 3101–10. doi:10.1182/blood-2013-05-502203. PMID 23982171.
  13. ↑ Wang RC, Chang ST, Hsieh YC, Huang WT, Hsu JD, Tseng CE; et al. (2014). "Spectrum of Epstein-Barr virus-associated T-cell lymphoproliferative disorder in adolescents and young adults in Taiwan". Int J Clin Exp Pathol. 7 (5): 2430–7. PMC 4069894. PMID 24966953.
  14. ↑ "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
  15. ↑ Doeden K, Molina-Kirsch H, Perez E, Warnke R, Sundram U (2008). "Hydroa-like lymphoma with CD56 expression". J Cutan Pathol. 35 (5): 488–94. doi:10.1111/j.1600-0560.2007.00836.x. PMID 17976208.
  16. ↑ Boddu D, George R, Nair S, Bindra M, G Mathew L (2014). "Hydroa Vacciniforme-Like Lymphoma: A Case Report From India". J Pediatr Hematol Oncol. doi:10.1097/MPH.0000000000000221. PMID 25072366.
  17. ↑ Plaza JA, Sangueza M (2015). "Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma". Am J Dermatopathol. 37 (1): 20–5. doi:10.1097/DAD.0000000000000158. PMID 25162933.
  18. ↑ "Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma".
  19. ↑ 19.0 19.1 "Thalidomide for the treatment of hydroa vacciniforme-like lymphoma:Report of four pediatric cases from Peru".
  20. ↑ "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
  21. ↑ "Epstein–Barr Virus-Associated Hydroa Vacciniforme-like Cutaneous Lymphoma in Seven Chinese Children".
  22. ↑ Li HY, Wang HL, Gao TZ, Zhuo ZH, Li DM, Li HX (2009). "[Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review]". Zhongguo Dang Dai Er Ke Za Zhi. 11 (7): 596–8. PMID 19651001.


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