BACKGROUNDMycosis fungoides (MF), characterized by a monoclonal proliferation of CD4-positive T cells, is the most common type of cutaneous T-cell lymphoma (CTCL) and constitutes almost 50% of all primary cutaneous lymphomas. 1 According to a newly published study, the incidence of MF increased from 3.0 per million person-years in the 1970s to 5.9 in the 2010s, 2 with a higher incidence in Blacks. 3 MF generally affects adult or elderly patients with a male-to-female ratio of 1.6-2.0:1. 1 Cases of children and adolescents, however, have also been reported. 4,5 Patients
Limited data are available about the underlying causes of hemophagocytic lymphohistiocytosis (HLH) in adults. We collected and analyzed the data of 555 cases of adult HLH. HLH in 242 patients were malignancies-related and lymphoid malignancies (42.0%, 233/555) were the most common causes. Aggressive natural killer-cell leukemia, diffuse large B-cell lymphoma, and extranodal natural killer/T-cell lymphoma, nasal type were the most common specified pathological subtypes. Epstein-Barr virus (EBV) (69.0%, 100/145) was the most common pathogen among the cases of infections-related HLH (26.1%, 145/555). Malignancies-related HLH showed male preponderance, more common splenomegaly, more severe anemia and thrombocytopenia, and significantly elevated soluble CD25. In patients with abnormal lymphoid cells in the bone marrow (BM) and increased EBV DNA copy number, 48.9% (45/92) of them were aggressive natural killer-cell leukemia. In patients with abnormal lymphoid cells in the BM and normal EBV DNA copy number, 66.2% (47/71) of them were B-cell non-Hodgkin lymphoma. In patients with elevated EBV DNA copy number but no abnormal lymphoid cells in the BM, 71.0% (98/138) of these cases were EBV infection. In conclusion, lymphoid malignancy is the most common underlying cause of adult HLH, followed by EBV infection. Based on the BM morphology and EBV load, we developed a diagnostic flow for rapid determination of the triggers for HLH.
Background: Extranodal natural killer/T-cell lymphoma (NKTCL), nasal type mostly involves the upper aerodigestive tract (UAT). NKTCLs derived from the UAT are referred to as nasal NKTCLs, while those without UAT involvement are referred to as extra-nasal NKTCLs. In this study, we aimed to investigate the outcomes and survival trends of NKTCLs from different anatomical sites.Methods: Data from the US Surveillance, Epidemiology, and End Results (SEER) database on NKTCL (diagnosed between 1987 and 2016) were retrospectively analyzed.Results: A total of 714 patients with NKTCL were included. The median overall survival (OS) and cancerspecific survival (CSS) were 36 and 57 months, respectively. For the entire cohort, the OS was improved from era 1 to era 2 with marginal significance (P=0.0595), however, no improvement was shown in CSS.For nasal NKTCLs, the OS of patients from era 2 was significantly improved compared to that of patients from era 1 (P=0.0244). The OS was significantly improved in non-cavity nasal NKTCLs (P=0.031) but not in nasal cavity NKTCLs (P=0.2982). Significant improvements in OS (P=0.0025) and CSS (P=0.0176) were found in stage I/II non-cavity nasal NKTCLs. For patients with extra-nasal NKTCLs, no difference was found in survival outcomes between the 2 eras. Conclusions: We have demonstrated that the outcomes of non-cavity nasal NKTCLs, especially those in stage I/II, have improved in the new era, while the outcomes of nasal cavity NKTCLs and extra-nasal NKTCLs have not improved. Our study highlights the heterogeneity in clinical outcomes and biology among NKTCLs from different sites. More studies are warranted to define the optimal treatments for patients with NKTCLs.
Purpose: To evaluate the role of circulating Epstein-Barr virus (EBV) DNA in lymphoma-associated hemophagocytic lymphohistiocytosis.Methods: We retrospectively analyzed 306 adult patients with lymphoma-associated hemophagocytic lymphohistiocytosis admitted to the First A liated Hospital of Nanjing Medical University from August 2009 to November 2022.Results: T/NK-cell malignancies (54.3%, 166/306) were the most common subtypes, followed by B-cell non-Hodgkin lymphoma (38.2%, 117/306). Elevated whole-blood EBV DNA was observed in 55.8% (164/294) of the patients and the median number was signi cantly higher in the T/NK malignancies (199500, than that in the B-cell non-Hodgkin lymphoma (5520, 1240-28400, P < 0.001).The optimum cutoff value for the overall survival of EBV DNA was determined as 43600 copies/mL. Compared to the patients with EBV DNA ≤ 43600 copies/mL, those with EBV DNA > 43600 copies/mL were younger and had more T/NK-cell malignancies, more bone marrow in ltration, lower levels of neutrophils and brinogen, and higher levels of alanine aminotransferase, aspartate aminotransferase, lactic dehydrogenase, triacylglycerol, and β 2 -microglobulin. A higher load of EBV DNA (> 43600 copies/mL), thrombocytopenia (< 100×10 9 /L), neutropenia (< 1×10 9 /L), hypo brinogenemia (≤ 1.5 g/L), and elevated levels of creatinine (> 133 μmol/L) were independent adverse predictors of overall survival. A prognostic index based on EBV DNA and the other four factors was established to categorize the patients into four groups with signi cantly different outcomes. Conclusion:Our study identi ed high EBV load as a risk factor for lymphoma-associated HLH and established a prognostic index based on EBV DNA to predict patients' outcomes.
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