Hydroa vacciniforme-like lymphoma
Jump to navigation
Jump to search
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
- CD8 positive[6]
- Some cases are CD4 positive[7]
- Always express granzyme B and T-cell intracellular antigen 1 (TIA-1) independently of the cell type derivation[8]
- CD45RO or CD56 positive[4]
- Monoclonal-type rearrangement of the T-cell receptor gamma[1]
- CD30 positive[9]
Differential Diagnosis
- 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
- Lupus[10]
- Herpes simplex[11][1]
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
- Skin lesions may or may not appear in sunlight exposed areas:[15]
- Typical B symptoms[16]
- Periorbital swelling[17]
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
- No significant beneficial results have been achieved with chemotherapy and radiotherapy.[19][20]
- Thalidomide has been recently used for the treatment of HVLL.[19]
- Skin eruptions can be treated with intramuscular interferon alfa.[21]
- Glucocorticoids can successfully relieve symptoms associated with HVLL.[22]
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 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.
- ↑ 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".
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 12.0 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.
- ↑ 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.
- ↑ "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
- ↑ 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.
- ↑ "Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma".
- ↑ 19.0 19.1 "Thalidomide for the treatment of hydroa vacciniforme-like lymphoma:Report of four pediatric cases from Peru".
- ↑ "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
- ↑ "Epstein–Barr Virus-Associated Hydroa Vacciniforme-like Cutaneous Lymphoma in Seven Chinese Children".
- ↑ 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.