Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases
Abstract Background Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia. It can progress to systemic lymphoma. However, prognostic factors and treatment rema...
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| Format: | Article |
| Language: | English |
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BMC
2019-07-01
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| Series: | Diagnostic Pathology |
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| Online Access: | http://link.springer.com/article/10.1186/s13000-019-0859-4 |
| _version_ | 1818097235525632000 |
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| author | Na Guo Yueqiong Chen Yu Wang Yuhua Huang Yanfen Feng Min Li Huilan Rao |
| author_facet | Na Guo Yueqiong Chen Yu Wang Yuhua Huang Yanfen Feng Min Li Huilan Rao |
| author_sort | Na Guo |
| collection | DOAJ |
| description | Abstract Background Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia. It can progress to systemic lymphoma. However, prognostic factors and treatment remain unclear. Methods This retrospective study reviewed the clinical, morphologic, immunophenotypical features, and clinical treatment of 19 patients with HV-LPD. Results All 19 patients had skin lesions in the face, extremities, or areas unexposed to the sun, including edema, blistering, ulceration, and scarring. The course was slowly progressive and relapsing. Histopathology showed an atypical lymphocytic infiltrate in the dermis and/or subcutaneous tissue. The lesions had a cytotoxic T/NK-cell immunophenotype. Among 19 patients, 7 (37%) exhibited CD4+ T cells, 5 (26%) exhibited CD8+ T cells, and 7 (37%) exhibited CD56+ cells. Of 12 cases with a T-cell phenotype, molecular analyses demonstrated that 7 had monoclonal rearrangements in the T-cell receptor genes. Three cases had an NK-cell phenotype and had polyclonal rearrangements in the TCR genes. All cases were associated with EBV infections. Among 19 patients, 9 (47.4%) received chemotherapy. Only one patient received allogeneic transplantation and EBV-specific cytotoxic T lymphocyte treatment after chemotherapy. That patient was the only one alive without disease at the latest follow up. Nine patients died of systemic lymphoma with disease progression, indicating irreversible process. Conclusions This study confirmed that HV-LPD is a broad-spectrum EBV+ lymphoproliferative disorder. It progressed to EBV+ systemic T/NK lymphoma, although some patients had a more indolent, chronic course. Cytopenia, elevated lactate dehydrogenase, destructive-multiorgan involvement, and older age were poor prognostic factors. Only allogeneic transplantation was curative. |
| first_indexed | 2024-12-10T23:17:18Z |
| format | Article |
| id | doaj.art-377769614af34633ad8e72e56c0f8ba2 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-10T23:17:18Z |
| publishDate | 2019-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-377769614af34633ad8e72e56c0f8ba22022-12-22T01:29:48ZengBMCDiagnostic Pathology1746-15962019-07-0114111110.1186/s13000-019-0859-4Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 casesNa Guo0Yueqiong Chen1Yu Wang2Yuhua Huang3Yanfen Feng4Min Li5Huilan Rao6State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineState Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer MedicineAbstract Background Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia. It can progress to systemic lymphoma. However, prognostic factors and treatment remain unclear. Methods This retrospective study reviewed the clinical, morphologic, immunophenotypical features, and clinical treatment of 19 patients with HV-LPD. Results All 19 patients had skin lesions in the face, extremities, or areas unexposed to the sun, including edema, blistering, ulceration, and scarring. The course was slowly progressive and relapsing. Histopathology showed an atypical lymphocytic infiltrate in the dermis and/or subcutaneous tissue. The lesions had a cytotoxic T/NK-cell immunophenotype. Among 19 patients, 7 (37%) exhibited CD4+ T cells, 5 (26%) exhibited CD8+ T cells, and 7 (37%) exhibited CD56+ cells. Of 12 cases with a T-cell phenotype, molecular analyses demonstrated that 7 had monoclonal rearrangements in the T-cell receptor genes. Three cases had an NK-cell phenotype and had polyclonal rearrangements in the TCR genes. All cases were associated with EBV infections. Among 19 patients, 9 (47.4%) received chemotherapy. Only one patient received allogeneic transplantation and EBV-specific cytotoxic T lymphocyte treatment after chemotherapy. That patient was the only one alive without disease at the latest follow up. Nine patients died of systemic lymphoma with disease progression, indicating irreversible process. Conclusions This study confirmed that HV-LPD is a broad-spectrum EBV+ lymphoproliferative disorder. It progressed to EBV+ systemic T/NK lymphoma, although some patients had a more indolent, chronic course. Cytopenia, elevated lactate dehydrogenase, destructive-multiorgan involvement, and older age were poor prognostic factors. Only allogeneic transplantation was curative.http://link.springer.com/article/10.1186/s13000-019-0859-4Epstein-Barr virus-positiveHydroa vacciniforme-like lymphoproliferative disorderClinicopathologic featuresPrognostic factorsTreatment |
| spellingShingle | Na Guo Yueqiong Chen Yu Wang Yuhua Huang Yanfen Feng Min Li Huilan Rao Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases Diagnostic Pathology Epstein-Barr virus-positive Hydroa vacciniforme-like lymphoproliferative disorder Clinicopathologic features Prognostic factors Treatment |
| title | Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases |
| title_full | Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases |
| title_fullStr | Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases |
| title_full_unstemmed | Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases |
| title_short | Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases |
| title_sort | clinicopathological categorization of hydroa vacciniforme like lymphoproliferative disorder an analysis of prognostic implications and treatment based on 19 cases |
| topic | Epstein-Barr virus-positive Hydroa vacciniforme-like lymphoproliferative disorder Clinicopathologic features Prognostic factors Treatment |
| url | http://link.springer.com/article/10.1186/s13000-019-0859-4 |
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