Hydroa vacciniforme-like lymphoma

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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]Although rare, it may present in adult population.

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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