Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases.

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.

Increased lymphocyte to monocyte ratio is associated with better prognosis in patients with newly diagnosed metastatic nasopharyngeal carcinoma receiving chemotherapy.

Inflammation has been demonstrated to be widely involved in the carcinogenesis of nasopharyngeal carcinoma (NPC). However, the prognostic significance of lymphocyte to monocyte ratio (LMR) in metastatic NPC is not fully addressed. The purpose of the study is to investigate the prognostic impact of pre-treatment absolute lymphocyte count (ALC), absolute monocyte count (AMC), and LMR on patients with newly diagnosed metastatic NPC undergoing chemotherapy. Between January 2006 and December 2010, patients with newly diagnosed metastatic NPC undergoing chemotherapy were retrospectively collected. The prognostic significance of baseline clinical features and inflammatory markers was investigated. A total of 256 patients were eligible for the study. The best cut-off value of ALC, AMC, and LMR was 2.25 × 10(9)/L, 0.35 × 10(9)/L, and 5.07, respectively. Patients in the high LMR group had a significantly longer overall survival (OS) (25.0 months [24.50-25.49]) than patients in the low LMR group (16.0 months [15.51-16.49]; p < 0.001). In addition, ALC ≥ 2.25 × 10(9)/L (HR, 0.59; 95% CI, 0.43-0.81; p = 0.001) and LMR ≥ 5.07 (HR, 0.42; 95% CI, 0.30-0.59; p < .001) remained as independent prognostic factors for superior OS, while AMC did not retained its prognostic significance in COX multivariate analysis. Pre-treatment ALC and LMR were demonstrated to be independent prognostic factors in patient with newly diagnosed metastatic NPC receiving chemotherapy. Future prospective studies are needed to validate the findings.