Hematological Oncology最新文献

Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL), a recently defined entity in WHO-HAEM5, includes primary diffuse large B-cell lymphoma (DLBCL) occurring in immune-privileged areas like the central nervous system (PCNS-LBCL), vitreoretinal system (PVR-LBCL), and testis (PT-LBCL) in immunocompetent patients. This study aimed to identify prognostic factors and create a predictive model for IP-LBCL. We analyzed 213 newly diagnosed IP-LBCL patients from April 2006 to April 2023. A nomogram and prognostic index, IPLBCL-PI, were developed based on elevated LDH, ECOG ≥ 2, and PCNS-LBCL subtype as independent risk factors for poorer PFS. IPLBCL-PI categorized patients into four risk groups: low, low-intermediate, intermediate-high, and high. The model effectively predicted both PFS and OS in the training cohort and was validated in two external centers. Subgroup analyses showed that IPLBCL-PI outperformed the Nottingham/Barcelona (NB) and Memorial Sloan Kettering Cancer Center (MSKCC) models in PCNS-LBCL and was comparable to the International Prognostic Index (IPI) in PT-LBCL. IPLBCL-PI is the first prognostic model for IP-LBCL, offering risk stratification and aiding clinical decision-making for this rare entity.

Our study aimed to assess the prognostic significance of the interim National Comprehensive Cancer Network International Prognostic Index and PET-CT-related parameters for predicting patient outcomes and achieving precise risk stratification for diffuse large B-cell lymphoma (DLBCL) patients. We retrospectively analyzed the clinicopathological and PET-CT data of 498 patients diagnosed with DLBCL across three medical centers in China. 418 patients were eligible for subsequent analysis after excluding those with incomplete data and 70% of which were randomly selected as the discovery cohort, whereas the remaining 30% constituted the validation cohort. The impact of candidate factors on survival was assessed via univariate and multivariate Cox proportional hazards models. The area under the curve AUC and C-index were calculated to assess the predictive performance of models. Univariate and multivariate Cox regression analyses identified changes in total lesion glycolysis (ΔTLG), iNCCN-IPI, interim abdominal residual disease (iARD) status, and changes in the maximum standardized uptake value (ΔSUVmax) as independent prognostic factors. Leveraging the outcomes of the multivariate analysis, we constructed the iPET-NCCN-IPI prognostic model and categorized DLBCL patients into two separate prognostic risk groups based on their computed Risk Scores (RS = 0.90×iNCCN-IPI + 1.41×ΔTLG + 0.79×ΔSUVmax + 0.83×iARD). The predictive performance of the model was validated by calculating the area under the receiver operating characteristic curve and the C-index. Notably, compared with other models, the iPET-NCCN-IPI demonstrated superior prognostic capability. In conclusion, our study indicates that the iPET-NCCN-IPI stratifies DLBCL patients into two distinct prognostic risk groups and surpasses other models in prognostic predictive ability.

Low-dose venetoclax plus strong CYP3A4 inhibitor voriconazole were commonly used for acute myeloid leukemia (AML) patients who were unfit for intensive chemotherapy in China. However, the efficacy and safety of this schedule have not been well investigated. We analyzed clinical data from 54 patients with a median age of 67 years. Thirty patients received a standard dose of venetoclax plus azacitidine (cohort 1), whereas another 24 patients received low-dose venetoclax plus voriconazole plus azacitidine (cohort 2). The composite complete remission (complete remission or complete remission with incomplete hematologic recovery; CR/CRi) rate was 76.7% (23/30) in cohort 1 and 87.5% (21/24) in cohort 2 (p = 0.483). At a median follow-up of 16 months, the median progression-free survival was 12 months in cohort 1 and 18 months in cohort 2 (p = 0.241). The median overall survival was 14 months in cohort 1 and 19 months in cohort 2 (p = 0.453). Key adverse events included cytopenia and infections. Grade 3 or higher infections occurred in 36.7% of the patients in cohort 1 and 20.8% of those in cohort 2. In conclusion, this study demonstrated the safety and effectiveness of the combination of low-dose venetoclax and voriconazole in unfit AML.

This study aimed to discuss the clinical outcomes of extramedullary multiple myeloma in the era of novel agents, based on the largest dataset regarding extramedullary multiple myeloma in China. This study included 597 patients without extramedullary disease (EMD) (non-EMD), 324 with extramedullary bone-related disease (EMB) and 138 with de novo extramedullary extraosseous disease (EME). There were no significant differences in overall survival (OS, p = 0.638) or progression-free survival (PFS, p = 0.195) between non-EMD and EMB patients. However, de novo EME patients exhibited significantly worse OS (p < 0.01) and PFS (p < 0.01) compared to both EMB and non-EMD groups. Among non-EMD and EMB patients, those with ≥ 2 high-risk cytogenetic abnormalities (HRA) experienced extremely poor prognoses, categorizing them as ultra-high-risk multiple myeloma. Similarly, de novo EME patients with ≥ 1 HRA demonstrated very poor outcomes and should also be considered ultra-high risk. Notably, single transplantation was shown to mitigate the adverse prognosis of de novo EME patients. Furthermore, the daratumumab bortezomib lenalidomide dexamethasone (DVRD) quadruplet regimen showed potential as effective frontline therapies for de novo EME patients, offering hope for improved treatment outcomes in this challenging subgroup. These findings suggest that de novo EME represents an extremely poor prognosis and should be treated as a distinct entity within the multiple myeloma population. Furthermore, the results indicate that EMB may need to be excluded from the current EMD definition to better delineate these subgroups and guide therapeutic strategies.

The characteristics at diagnosis and clinical course of primary extranodal follicular lymphoma (EFL) have not been extensively described. The International Extranodal Lymphoma Study Group (IELSG) conducted an international retrospective survey aimed to describe the clinical features at diagnosis and the outcomes of FL cases with a clinically dominant extranodal component. The dataset included 605 pathologically reviewed cases from 19 different countries, and their outcomes were compared to those of nodal follicular lymphomas. The two most common presentation sites for EFL were the skin (n = 334) and the gastrointestinal tract (n = 72), with 22 cases having primary duodenal localization. These subsets exhibited unique features at diagnosis and significantly different overall survival (OS) patterns. After a median follow-up of 5.5 years, primary cutaneous lymphomas showed a superior outcome [10-year OS: 89% (95% CI, 83%–93%)], while primary gastrointestinal lymphomas had an intermediate outcome [10-year OS: 79% (95% CI, 59%–90%)]. Among the gastrointestinal lymphomas, primary duodenal lymphomas tended toward the best outcome [10-year OS: 95% (95% CI, 69%–99%)]. Other primary extranodal sites had inferior outcomes [10-year OS: 59% (95% CI, 48%–68%)], similar to primary nodal lymphomas [10-year OS: 57% (95% CI, 49%–64%)]. These findings support the identification of specific primary FL localizations as distinct entities with particular clinical and biological characteristics.