Hydroa vacciniforme-like lymphoma: Difference between revisions

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== Epidemiology and Demographics ==
== Epidemiology and Demographics ==
The main demographic group at risk is the pediatric population. Its seen mainly in children and adolescents from asia, central and south america and mexico. As well as other [[NK-cell lymphoma]]s, EBV-positive lymphomas are associated with a deficient ctytotoxic immune response to [[EBV]]<ref>{{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>. Adults are rarely affected.
 
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>


== Risk Factors ==
== Risk Factors ==

Revision as of 21:31, 23 February 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

Overview

Hydroa vacciniforme-like lymphoma is a EBV-positive peripheral T-cell lymphoma (Non-Hodgkin's lymphoma), that affects primarily pediatrics patients and has a bad prognosis.[1] It is associated with photosensitivity and insect bites.[2] Clinically, it manifests with vacciniform vesicles, necrotic ulcers and scars.[3]

Historical Perspective

Classification

Pathophysiology

Causes

Differential Diagnosis

Epidemiology and Demographics

Hydroa vacciniforme-like lymphoma occurs mainly in the pediatric population from South America, Central America and Asia.[4]

Risk Factors

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

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.[5] Patients may present with typical B symptoms,[6] which in some cases, are preceded by periorbtal swelling.[7]

Treatment

References

  1. 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. 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. 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.
  5. 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.
  6. 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.
  7. 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.


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