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. | |||
== Risk Factors == | == Risk Factors == | ||
Revision as of 21:22, 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
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 lymphomas, EBV-positive lymphomas are associated with a deficient ctytotoxic immune response to EBV[4]. Adults are rarely affected.
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
- ↑ 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.
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ "Hydroa vacciniforme-like lymphoma: a case report and literature review" (PDF).
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ 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.
- ↑ 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.
- ↑ 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.