Hydroa vacciniforme-like lymphoma
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 is a EBV-positive peripheral T-cell lymphoma (Non-Hodgkin's lymphoma), that affects primarily pediatric 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] Some cases of Hydroa vacciniforme-like lymphoma can have NK-cell surface receptors.[4]
Historical Perspective
Hydroa vacciniforme-like lymphoma reports date back from 1986,[5] although only since 2008, the WHO recognizes the HVLL as a separate entity from the Hydroa vacciniforme and classified it under "EBV positive T-cell lymphoproliferative disorders of childhood".[2]
Classification
Pathophysiology
Histopathology
Histopathologic examination showed lymphocytic infiltrate in the skin with the following characteristics:[6]
- Angiotropism
- Angiocentricity
Immunophenotype
- CD8 positive,[6] although some cases have been CD4 positive[7]. Indepently of the cell type derivation, they always express granzyme B and T-cell intracellular antigen 1 (TIA-1).[8]
- CD45RO or CD56.[4]
- Monoclonal-type rearrangement of the T-cell receptor gamma.[1]
Causes
Differential Diagnosis[1]
- Extranodal T-/NK-cell lymphoma, nasal type
- Mycosis fungoides
- Precursor T-cell lymphoblastic lymphoma
- Peripheral T-cell lymphoma, not otherwise characterized
- Primary cutaneous anaplastic large cell lymphoma
- Subcutaneous panniculitis-like T-cell lymphoma
Epidemiology and Demographics
Hydroa vacciniforme-like lymphoma occurs mainly in the pediatric population from South America, Central America and Asia.[9] Although rare, it may present in adult population.[10]
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.[11] Patients may present with typical B symptoms,[12] which in some cases, are preceded by periorbtal swelling.[13]
Treatment
References
- ↑ 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.
- ↑ 2.0 2.1 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ "Hydroa vacciniforme-like lymphoma: a case report and literature review" (PDF).
- ↑ 4.0 4.1 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.
- ↑ Oono T, Arata J, Masuda T, Ohtsuki Y (1986). "Coexistence of hydroa vacciniforme and malignant lymphoma". Arch Dermatol. 122 (11): 1306–9. PMID 3490832.
- ↑ 6.0 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.
- ↑ 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.
- ↑ "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; 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.
- ↑ 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.
- ↑ 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.