Primary Cutaneous Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder-2 Cases With Unusual, Prolonged Clinical Course

Park, Sanghui; Lee, Dong Youn; Kim, Won Seog; Ko, Young Hyeh

doi:10.1097/dad.0b013e3181d68381

Cited by 20 publications

(13 citation statements)

References 23 publications

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“…This is reinforced by a recent description by another group of 2 similar cases lacking facial involvement. 44 Overall, these data suggest that the spectrum of cutaneous EBV-positive lymphoproliferative disorders may be larger than initially suspected, also including cases with features of HMB. 29,55 Collectively, these cases could be termed Endemic Childhood Cutaneous NK/T-cell Lymphoma, thereby emphasizing their common distinctive features.…”

Section: Discussionmentioning

confidence: 77%

EBV-associated Cutaneous NK/T-cell Lymphoma

Rodríguez‐Pinilla¹,

Barrionuevo²,

Garcı́a

et al. 2010

American Journal of Surgical Pathology

102

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We have reviewed clinically, morphologically, and immunophenotypically a series of 14 Epstein-Bar virus (EBV)+ cutaneous natural killer cell (NK)/T-cell lymphoma from Peru. Most (11 out of 14) of these cases fit well into the category of Hydroa vacciniforme-like lymphoma (HVLL), but 3 have a different clinical presentation, without facial involvement. In all 14 cases, skin lesions present in both the sun-exposed and nonexposed areas exhibited a slowly progressive relapsing course, changing from edema, to blistering, ulceration, and final scarring. The immunophenotype had a cytotoxic T or NK-cell lineage. The mean time of disease before admission to hospital was 69 months (range, 6 mo to 31 y). Only 2 patients had fever, hepatosplenomegaly, systemic lymphadenopathy, and a high lactate dehydrodenage (LDH) level at the time of diagnosis, whereas 10 had facial swelling. After treatment, only 4 patients remain alive, although with persistent disease. Ten patients died after a mean follow-up of 11.6 months after the initial diagnosis (range, 1 to 32 mo), because of concurrent infections (4 cases), disease progression (4 patients) or both (2 patients). Endemic Epstein-Bar virus (EBV)-positive cutaneous NK/T-cell lymphoproliferative disorders in childhood and early adulthood are characterized by a protracted clinical course, eventually leading to an aggressive phase characterized by concurrent infections and disease progression.

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Section: Discussionmentioning

confidence: 77%

EBV-associated Cutaneous NK/T-cell Lymphoma

Rodríguez‐Pinilla¹,

Barrionuevo²,

Garcı́a

et al. 2010

American Journal of Surgical Pathology

102

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“…(6)(7)(8)(9) However, the definition of each EBV-associated T/NK LPD is unclear and there is significant overlap between them. (5,(9)(10)(11)(12)(13) Therefore, diagnosis of EBV-associated T/NK LPD can be problematic.…”

Section: Cd8mentioning

confidence: 99%

Application of flow cytometric in situ hybridization assay to Epstein–Barr virus‐associated T/natural killer cell lymphoproliferative diseases

et al. 2012

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Epstein–Barr virus (EBV) infects various types of lymphocytes and is associated with not only B cell‐origin lymphoma, but also T or natural killer cell lymphoproliferative diseases (T/NK LPD). Recently, we established a novel assay to identify EBV‐infected cells using FISH. Using this assay, dual staining with antibodies to both surface antigens and an EBV‐encoded small RNA (EBER) probe can be performed. In the present study, we applied this recently developed FISH assay to EBV‐associated T/NK LPD to confirm its diagnostic utility. Using FISH, we prospectively analyzed peripheral blood from patients with suspected EBV‐associated T/NK LPD. The results were compared with those obtained using immunobead sorting followed by quantitative PCR. In all, 26 patients were included study. Using FISH, 0.15–67.0% of peripheral blood lymphocytes were found to be positive for EBER. Dual staining was used to determine EBER‐positive cell phenotypes in 23 of 26 subjects (88.5%). In five of seven patients with hydroa vacciniforme‐like lymphoma (an EBV‐positive cutaneous T cell lymphoma), EBER‐positive cells were identified as CD3+CD4−CD8− TCRγδ+ T cells. Furthermore, in a 25‐year‐old male patient with systemic EBV‐positive T cell LPD, two lymphocyte lineages were positive for EBER: CD4+CD8− and CD4−CD8+T cells. Thus, we confirmed that our newly developed assay is useful for quantifying and characterizing EBV‐infected lymphocytes in EBV‐associated T/NK LPD and that it can be used not only to complement the pathological diagnosis, but also to clarify the pathogenesis and to expand the spectrum of EBV‐associated diseases. (Cancer Sci, doi: 10.1111/j.1349‐7006.2012.02305.x, 2012)

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“…So the latest edition of WHO classification grouped it as one of major types of EBV-positive T-cell lymphoproliferative disorders of childhood, and suggested "HV-like lymphoma" could be a more appropriate classification and descriptive term. 1 We have reviewed HV-like lymphoma reported in the English literature in recent 2 decades [2][3][4][5]8,[10][11][12][13][14] (Table 1). It revealed that all patients were from Asia and South America, especially Korea, Japan, China, and Mexico.…”

Section: Discussionmentioning

confidence: 99%

Primary Cutaneous Hydroa Vacciniforme–Like Lymphoma With Indolent Clinical Course

et al. 2012

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Primary cutaneous hydroa vacciniforme (HV)-like lymphoma is a rare, Epstein-Barr virus-associated cutaneous neoplasm characterized by photosensitive papulovesicular eruption and usually associated with poor prognosis. This report presents 2 cases of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 2 and 3 years, respectively. Both patients presented with erythema, papulovesicles, scars, ulcerations, and edema on the face and extremities. Skin biopsies revealed epidermal vesicle with small- to medium-sized atypical lymphoid cells infiltrating in the dermis and subcutis. The lymphoid cells were strongly immunoreactive to CD8 and CD56. The Epstein-Barr virus genomes were also found in both skin biopsies. By genetic analysis, one of patients showed T-cell receptor-γ gene clonal rearrangement. The patients underwent glucocorticoid treatment and obtained remarkable clinical improvement with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up. A long-term follow-up is suggested to be performed to inspect the progression for this tumor.

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Primary Cutaneous Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder-2 Cases With Unusual, Prolonged Clinical Course

Cited by 20 publications

References 23 publications

EBV-associated Cutaneous NK/T-cell Lymphoma

EBV-associated Cutaneous NK/T-cell Lymphoma

Application of flow cytometric in situ hybridization assay to Epstein–Barr virus‐associated T/natural killer cell lymphoproliferative diseases

Primary Cutaneous Hydroa Vacciniforme–Like Lymphoma With Indolent Clinical Course

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