Low prevalence and independent prognostic role of del(11q) in Chinese patients with chronic lymphocytic leukemia.

The 11q deletion (del(11q)) is a conventional cytogenetic aberration observed in chronic lymphocytic leukemia (CLL) patients. However, the prevalence and the prognostic value of del(11q) are still controversial. In this research, we retrospectively explored the prevalence, association, and prognostic significance of del(11q) in 352 untreated and 99 relapsed/refractory Chinese CLL patients. Totally 11.4% of untreated and 19.2% of relapsed/refractory patients harbored del(11q). Del(11q) was more common in patients with ß2-microglobulin > 3.5 mg/L, positive CD38, positive zeta-chain associated protein kinase 70, unmutated immunoglobulin heavy variable-region gene and ataxia telangiectasia mutated mutation. Kaplan-Meier method and univariate Cox regression indicated that del(11q) was an independent prognostic factor for overall survival (OS). Based on the results of univariate Cox regression analysis, two nomograms that included del(11q) were established to predict survival. Desirable area under curve of receiver operating characteristic curves was obtained in the training and validation cohorts. In addition, the calibration curves for the probability of survival showed good agreement between the prediction by nomogram and actual observation. In summary, the prevalence of del(11q) is relatively low in our cohort and del(11q) is an unfavorable prognostic factor for untreated CLL patients. Besides, these two nomograms could be used to accurately predict the prognosis of untreated CLL patients.

Prognostic nutritional index, a novel biomarker which predicts worse prognosis in diffuse large B cell lymphoma.

The prognostic nutritional index (PNI), an indicator of nutritional status and systemic inflammation, is associated with survival in several types of lymphoma. The purpose of this study was to investigate the prognostic value of PNI in diffuse large B cell lymphoma (DLBCL). With three hundred and ten patients were enrolled, the median level of PNI was 45.90 (range 25.30-139.70). According to the receiver operating characteristic (ROC) curve, 44.85 was determined to be the best cutoff value to divide patients into two different groups. With a median follow-up of 33.3 months (range 3.5-118.5), compared with the high PNI group, the 3-year and adjusted 3-year progression-free survival (PFS) and overall survival (OS) were worse in the low PNI group (all P < 0.050). Multivariate Cox analysis suggested that low PNI was an independent risk factor for PFS (hazard ratio (HR) 2.196, 95 % CI 1.197-4.030, P = 0.011) and showed a tendency to predict inferior OS (HR 1.918, 95 % CI 0.932-3.948, P = 0.077). Furthermore, PNI combined with other significant prognostic factors to build a novel prognostic index, namely NPI, was more accurate than the National Comprehensive Cancer Network international prognostic index (NCCN-IPI) to predict worse PFS and had a similar effect on predicting OS. All these findings suggested that PNI, as a novel available biomarker, was of prognostic significance in DLBCL patients.

Time-limited, Combined Regimen in Chronic Lymphocytic Leukemia: A Promising Strategy to Achieve a Drug Holiday.

Chemoimmunotherapy (CIT) is defined as standard first line treatment for chronic lymphocytic leukemia (CLL) patients while patients with unfavorable biological characteristics such as unmutated immunoglobulin heavy chain (UM-IGHV) and TP53 aberration failed to benefit from it. The emergency of the small molecular targeted agents including Bruton's tyrosine kinase (BTK) inhibitor (BTKi) leads to a brand-new era, from a CIT to a chemo-free era in CLL. However, the treatment of target agents is not enough to attain a deep remission and high rate of complete remission (CR), especially in patients with high risks. The long duration brought about problems, such as cost, drug resistance and toxicity. To benefit CLL in progression free survival (PFS) and long-term remission, exploration of time-limited therapies, mainly with BTKi plus CIT and BCL2i based combination therapy has become a mainstream in clinical trials. The time-limited combination therapy shed light on the promising potentiality to attain sustainable deep remission and partly overcame the risk factors, although long term follow-up is required to consolidate the conclusion. In this review, we intend to introduce key results of clinical trials with combination therapy, discuss the achievements and limitations and put forward future direction for clinical trial design in this field.

Pretreatment whole blood Epstein-Barr virus DNA predicts prognosis in Hodgkin lymphoma.

The study investigated pretreatment Epstein-Barr virus (EBV) DNA status and its prognostic values in 96 patients newly diagnosed Hodgkin lymphoma (HL). With 13.5 % patients in positive EBV DNA status before therapy, the positive group had inferior progression-free survival (PFS) (P = 0.023) as well as overall survival (OS) (P = 0.001). Pretreatment EBV DNA positivity was observed as an independent prognostic factor in OS (P = 0.036) while a trend to predict PFS (P = 0.064). By monitoring changes of EBV DNA copies in 13 patients with positive pretreatment EBV DNA status, 5 of 6 patients with complete response (CR) had their copies undetectable after 3 cycles of first-line treatment and 7 patients with progressive disease (PD) all had elevated EBV DNA copies during their relapsed period. Whole blood EBV DNA may be an adjunctive biomarker to reflect treatment response, risk of disease relapse as well as prognosis in HL patients.

25-Hydroxy vitamin D deficiency predicts inferior prognosis in Hodgkin lymphoma.

The study investigated serum 25-Hydroxy vitamin D (25-(OH)D) deficiency and its prognostic values of patients newly diagnosed Hodgkin lymphoma (HL). With seventy-seven patients enrolled, the median level of 25-(OH)D was 44.5 nmol/L (range, 15.5-100.9 nmol/L) and 16 (20.8 %) of them were considered as 25-(OH)D deficiency. With a median follow-up of 28 months (range, 4-56 months), the 2-year progression-free survival (PFS) and overall survival (OS) rate were 75.3 %±5.5 % and 94.7 %±3.0 %, respectively. Patients with deficient 25-(OH)D level had inferior PFS (P<0.001) as well as OS (P<0.001). In multivariate Cox analysis, 25-(OH)D deficiency was observed as an independent prognostic factor for both PFS (hazard ratio (HR) 3.323, 95 % CI 1.527-7.229, P = 0.002) and OS (HR 5.819, 95 % CI 1.322-25.622, P = 0.020). Receiver-operator characteristic (ROC) curve showed International Prognostic Score (IPS) plus 25-(OH)D deficiency (IPS-D) predicted more accurately than IPS in PFS (AUC: 0.735 (95 % CI 0.622-0.829) vs. 0.701 (95 % CI 0.586-0.800), P = 0.033) and OS (AUC: 0.864 (95 % CI 0.767-0.932) vs. 0.825 (95 % CI 0.722-0.902), P = 0.028). All these findings suggest that serum 25-(OH)D level may be an adjunctive indicator to predict prognosis in HL patient.

Circulating low CD4+/CD8+ ratio is associated with poor prognosis in Waldenstrom macroglobulinemia patients.

Waldenstrom macroglobulinemia (WM) is a rare type of non-Hodgkin lymphoma with great heterogeneity, and the data of peripheral blood T-lymphocyte subsets in WM are limited. This study aimed to investigate the clinical correlation and distribution of circulating T-lymphocyte subsets in newly diagnosed WM patients. We retrospectively searched medical records for 86 newly diagnosed WM patients. Comparisons of the absolute CD3+ T-lymphocyte count (ACD3C), CD4+ T-lymphocyte count (ACD4C), CD8+ T-lymphocyte count (ACD8C), and CD4+/CD8+ T-lymphocyte ratio (CD4+/CD8+) as continuous parameters in different groups were calculated. Univariate and multivariate analyses were used to assess prognostic factors for overall survival (OS) and progression-free survival (PFS). Young patients (<65 years) had lower ACD8C levels and a higher CD4+/CD8+ ratio. And the lower level of ß2-microglobulin (<3 mg/L) was associated with a higher CD4+/CD8+ ratio. With a median follow-up of 25 months, the univariate survival analysis showed that CD4+/CD8+ ratio inversion (CD4+/CD8+<1.5) was associated with shorter OS and PFS, and multivariate analysis confirmed that inverted CD4+/CD8+ ratio could be an independent adverse prognostic factor for OS and PFS. Additionally, initial treatment with rituximab or bortezomib significantly improved the PFS and OS of CD4+/CD8+ inversion patients but did not affect normal CD4+/CD8+ patients. We show that low circulating CD4+/CD8+ ratio at diagnosis is an adverse prognostic factor in WM patients and that first-line therapy which included rituximab or bortezomib significantly improved PFS and OS for patients with CD4+/CD8+ ratio less than 1.5.

Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases.

PURPOSE: To explore the clinical features and immunological mechanisms of Castleman disease (CD) complicated with autoimmune diseases (AID). METHODS: We explored the prevalence and clinical manifestations of CD complicated with AID by reviewing clinical, pathological, and laboratory data of 40 CD patients retrospectively, and then explored abnormal immune mechanisms in the co-existence of the two entities by monitoring lymphocyte subsets in peripheral blood. RESULTS: Paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren's syndrome, myasthenia gravis, and psoriasis were found to be coexisted with CD in 9/40 (22.5%) patients with different sequence of onset. No bias in the clinical and histological type of CD was observed for the occurrence of AID. CD patients with AID were more likely to have skin and/or mucous membrane damage and pulmonary complications, and presented elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and positive autoantibodies than those without AID (p < 0.05). Deregulated cellular and innate immune responses as indicated by decreased CD3+ T cells and increased natural killer cells were observed in peripheral blood of CD patients with AID (p < 0.05). UCD patients with AID were successfully treated with surgery and immunosuppressive therapy. MCD complicated by AID relieved with immunosuppressors, cytotoxic chemotherapy, and rituximab. CONCLUSION: Systemic inflammation/immunological abnormalities and organ dysfunction were associated with the occurrence of AID in CD. Impairment of cellular and innate immunity may be a candidate etiology for the coexistence of the two entities.

Low serum cholesterol levels predict inferior prognosis and improve prognostic index scoring for peripheral T-cell lymphoma, unspecified.

Peripheral T-cell lymphomas, unspecified (PTCL-U) is a heterogeneous group of non-Hodgkin lymphomas, arising from the transformation of mature, post-thymic T-cells. Prognostic index for PTCL-U (PIT) is based on Europeans and may not be applicable for Chinese PTCL-U patients. Besides, low circulating cholesterol concentration is associated with elevated cancer incidence and mortality. The purpose of our study was to assess the prognostic value of serum lipid levels in PTCL-U and improve PIT. We screened the prognostic factors associated with progression-free survival (PFS) and overall survival (OS) by multivariate Cox regression analysis in ninety-one enrolled patients. The results showed that low-level high-density lipoprotein cholesterol (HDL-C) and low-density lipoprotein cholesterol (LDL-C) were associated with unfavorable OS. Furthermore, we developed a new risk model, PITC, based on low-level HDL-C, LDL-C and PIT. In Chinese PTCL-U, PITC was superior to PIT in PFS and OS. In conclusion, serum cholesterol levels may be good candidates for predicting prognosis in PTCL-U.

C-reactive protein-to-albumin ratio is an independent poor prognostic factor in newly diagnosed chronic lymphocytic leukaemia: A clinical analysis of 322 cases.

Chronic lymphocytic leukaemia is one of the most common types of adult leukaemia. Cancer-related systemic inflammation response has been characterized to correlate with therapeutic outcome in patients with cancer. The C-reactive protein-to-albumin (CRP/ALB) ratio (CAR), which is an inflammatory marker, has been reported as a novel prognostic factor in several cancers. The aim of our study was to evaluate the prognostic value of the CAR in patients with chronic lymphocytic leukaemia (CLL). We retrospectively reviewed the clinical characteristics of 322 newly diagnosed CLL patients, investigated the correlations among pretreatment CAR, treatment-free survival (TFS) and overall survival (OS), assessed the prognostic effect of the CAR to compare with other inflammation-related prognostic index by the area under the curve (AUC), and combined CAR and CLL-international prognostic index (CLL-IPI) together to improve the current prognostic system. The results showed that CAR was an independent prognostic factor for OS. Furthermore, the predictive and discriminatory capacity of CLL-IPI together with CAR level was superior to that of CLL-IPI alone for OS. In conclusion, serum CRP and ALB levels are both simple and easily accessible parameters, whose ratio CAR may be good candidates for predicting prognosis in the future clinical practice of CLL.

Gemcitabine, oxaliplatin and dexamethasone (GemDOx) as salvage therapy for relapsed or refractory diffuse large B-cell lymphoma and peripheral T-cell lymphoma.

Background Outcomes of relapsed or refractory diffuse large B-cell lymphoma (DLBCL) and peripheral T-cell lymphoma (PTCL) remain poor. The objective of this study was to evaluate the efficacy and safety of gemcitabine, oxaliplatin and dexamethasone (GemDOx) with or without rituximab as salvage therapy in patients with relapsed or refractory DLBCL and PTCL. Materials and Methods: We retrospectively reviewed patients with relapsed or refractory DLBCL and PTCL receiving GemDOx as salvage therapy between Jul 1, 2011, and Aug 31, 2017. Results: Thirty-three (57.9%) patients with relapsed or refractory DLBCL and 24 (42.1%) with PTCL were included in this study. The median age was 57 years (inter-quartile range 46-67). The overall response rate (ORR) in DLBCL was 48.5% with 27.3% of complete remission (CR), and the 2-year progression-free survival (PFS) and 2-year overall survival (OS) was 21% and 44%. In patients with PTCL, ORR was 50.0% with CR rate of 29.2%; the 2-year PFS and 2-year OS was 28% and 49%, respectively. Common grade 3-4 hematological adverse events were thrombocytopenia (26.3%), anemia (15.7%) and neutropenia (15.7%). Conclusion: With acceptable efficacy and good tolerability, GemDOx might be a new therapeutic option for relapsed or refractory DLBCL and PTCL.

Prognostic value of 25-hydroxy vitamin D in extranodal NK/T cell lymphoma.

25-hydroxy vitamin D [25-(OH)D] is widely used to determine vitamin D status in clinic. The aim of our study was to evaluate the prognostic value of 25-(OH)D in extranodal NK/T cell lymphoma (ENKTL). Ninety-three (93) ENKTL patients with available serum 25-(OH)D values were enrolled in our study. Vitamin D deficiency is defined as a 25-(OH)D below 50 nmol/L (20 ng/ml). Univariate and multivariate regression analyses were performed to determine independent risk factors for progression-free survival (PFS) and overall survival (OS). Receiver operator characteristic (ROC) curves were plotted, and corresponding areas under the curves (AUC) were calculated to estimate the accuracy of PINK-E (prognostic index of natural killer lymphoma added with Epstein-Barr virus-DNA status) and 25-(OH)D deficiency in ENKTL risk-stratification. Our results suggested that the vitamin D deficiency was an independent inferior prognostic factor for both PFS [hazard ratio (HR), 2.869; 95% confidence interval (CI), 1.540 to 5.346; P = 0.003] and OS (HR, 3.204; 95% CI, 1.559 to 6.583; P = 0.006) in patients with ENKTL. Additionally, we demonstrated that adding 25-(OH)D deficiency to PINK-E score system indeed has a superior prognostic significance than PINK-E alone for PFS [AUC: 0.796 (95% CI: 0.699 to 0.872) vs. 0.759 (95% CI: 0.659 to 0.841), P = 0.020] and OS [AUC: 0.755 (95% CI: 0.655 to 0.838) vs. 0.721 (95% CI: 0.618 to 0.809), P = 0.040]. In conclusion, our study proved that 25-(OH)D deficiency was associated with inferior survival outcome of ENKTL patients.

The prognostic roles of hypogammaglobulinemia and hypocomplementemia in newly diagnosed diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma (DLBCL) is the most frequent type of lymphoma. Our retrospective study included 553 newly diagnosed DLBCL patients from May 2009 to October 2019. The relationships between hypogammaglobulinemia, hypocomplementemia and progression-free survival (PFS) and overall survival (OS) were assessed. In our center, 19.0% of patients had hypogammaglobulinemia, and 7.7% had hypocomplementemia at diagnosis. Immunoglobulin and complement deficiencies were associated with advanced disease and displayed inferior PFS and OS. Then, we designed a new immunization cumulative prognostic score (ICPS) model to comprehensively clarify the effect of these two variables on prognosis. Multivariate analysis showed that ICPS was an independent prognostic indicator for inferior clinical outcomes (PFS: p = 0.007, OS: p = 0.003). Furthermore, the predictive effect of ICPS combined with the International Prognostic Index (IPI) was superior to that of IPI alone (PFS: p = 0.016, OS: p = 0.037). In conclusion, hypogammaglobulinemia and hypocomplementemia could be regarded as adverse prognostic indicators in DLBCL.

[The Clinical and Laboratory Characteristics of Adult Langerhans Cell Histiocytosis Patients and the Influencing Factors of Prognosis].

OBJECTIVE: To observe the clinical and laboratory characteristics of adult Langerhans cell histiocytosis(LCH) patients and to analyze the influencing factors of its prognosis. METHODS: The clinical and laboratory charac-teristics of 38 adult LCH patients treated in our hospital from January 2010 to August 2019 were retrospective analyzed, and the clinical prognosis of the patients was analyzed. RESULTS: The median age of 38 patients was 41 (21-65) years old, and the ratio of male and female was about 2∶1. Among 38 patients, 44.7% (17/38) were involved in multiple systems, and 31.6% (12/38) were involved in high-risk organs (including liver, lung, hematopoietic system or spleen). The bone involvement was the most common (21/38, 55.3%), and the most common clinical symptom was pain (19/38, 50.0%). The result of laboratory showed that anemia (4/38，10.5%), thrombocytopenia (1/38，2.6%), neutropenia (2/38，5.3%), lymphopenia (6/38，15.8%), monocytosis (11/38，28.9%), C-reactive protein increasing (6/21，28.6%), erythrocyte sedimentation rate increasing (10/18, 55.3%), and ferritin protein increasing (9/17, 55.3%). The median follow-up time was 53 months, and a total of 5 patients were died. The 10-year overall survival rate of patients with single-system involvement was 100%, which was significantly higher than that of patients with multiple-system involvement (70.1%) (P=0.0078). The prognosis of patients without risk-organ involvement was better than that of patients with risk-organ involvement (10-year overall survival rate: 100% vs 60.6%) (P=0.0007). Further analysis showed that in addition to multiple-system involvement and risk-organ involvement, the increase of peripheral blood monocyte cells and the increase of ferritin protein were also associated with poorer prognosis of the patients. CONCLUSION: The multiple system involve-ment and risk-organ involvement, the increasing of monocyte cells and the increasing of ferritin protein were the independent risk factors of adult LCH patients.

Chronic lymphocytic leukemia-associated paraneoplastic pemphigus: potential cause and therapeutic strategies.

Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms. The prognosis of CLL-associated PNP is dismal due to its refractory course and secondary infection and no standard treatment was recommended. We retrospectively reported six CLL with PNP cases from 842 cases of CLL including diagnosis, treatment and prognosis. The median time between the initial of CLL to PNP was 36 months while the median overall survival from the diagnosis of PNP was 26 months. And three cases died of lung infection while 5 developed pulmonary symptoms. And 5 cases received fludarabine-based chemotherapy before developing PNP, which suggesting fludarabine was one of potential causes of PNP. For the treatment, five patients were rescued by combined regimens including rituximab, methylprednisolone, immunoglobulin, fresh frozen plasma and the last received ibrutinib combined with short-term prednisone. Fludarabine-based regimen may be one of the potential causes of PNP. The combined regimen might shed a new light, while ibrutinib is a promising drug for CLL with PNP, but needs much more evidence. PNP should be carefully treated to guide early diagnosis and intervention for a better prognosis.

Chidamide, a histone deacetylase inhibitor, inhibits autophagy and exhibits therapeutic implication in chronic lymphocytic leukemia.

Novel agents have made the management of chronic lymphocytic leukemia (CLL) more promising and personalized. However, long-term treatment is still warranted which may result in toxicity and resistance. Thus, new combination therapy may help achieve deeper remission and limited-duration therapy. Histone deacetylase inhibitors (HDACi) can affect many tumors by modulating key biological functions including autophagy. Studies have shown that some novel targeted agents including ibrutinib induce autophagy. This study aimed to explore the effect of oral HDAC inhibitor, chidamide, on CLL cells as well as the role of autophagy in this process. Here, we showed that autophagy flux in CLL cells was inhibited by chidamide via post-transcriptional modulation and chidamide had cytostatic and cytotoxic effects on CLL cells. Besides, the pro-survival role of autophagy in CLL cells was validated by using autophagy inhibitor and knocking down critical autophagy gene. Notably, a combination of chidamide and ibrutinib showed significant synergism and downregulated ibrutinib-induced autophagy. This work highlights the therapeutic potential of chidamide via its effect on autophagy, especially in combination with ibrutinib.

[Roles of PET/CT in Predicting the Prognosis of Diffuse Large B Cell Lymphoma Patients Treated with Chimeric Antigen Receptor T Cell Therapy].

OBJECTIVE: To investigate the prognosis prediction value of PET/CT in DLBCL patients treated with CAR-T therapy. METHODS: The effects of PET/CT were retrospectively explored on 13 R/R DLBCL patients who were treated with CAR-T therapy. Parameters reflecting tumor metabolic burden, such as metabolic tumor volume (MTV) and total lesion glycolysis (TLG) were measured before and after CAR-T treatment. RESULTS: Patients with larger baseline MTV or longer sum of longest diameters showed shorter overall survival (OS) time than those with low tumor burden. Patients achieved complete remission (CR), partial remission (PR) and minor remission (MR) determined by response evaluation criteria in lymphoma (RECIL) in 12 weeks showed progression-free survival and OS time superior to those of patients with no remission. In addition, it was found that 2 patients with residual masses classified as PR by contrast-enhanced CT of patients were evaluated as complete metabolic response by PET/CT imaging. CONCLUSION: PET/CT shows a great value in the evaluation of prognosis and response in CAR-T-treated R/R DLBCL patients.

High viral loads of circulating Epstein-Barr virus DNA copy number in peripheral blood is associated with inferior prognosis in patients with mantle cell lymphoma.

Mantle cell lymphoma (MCL) is a distinct subtype of B cell non-Hodgkin lymphoma. No research has yet documented to investigate the prognostic implications of Epstein-Barr virus (EBV) infection in MCL. The objective of this study was to determine whether EBV DNA load may influence the heterogeneity in the course of the disease in MCL patients. Eighty-eight MCL patients were retrospectively enrolled in the study. EBV DNA load was detected by real-time quantitative PCR for quantification. The univariate and multivariate Cox proportional hazards models were established for the estimation of prognostic factors. Twenty-seven patients were detected positive for EBV DNA and the median virus titer was 1.72×104 copies/mL (range, 8.20×102 to 4.14×105 copies/mL). With a median follow-up of 39 months (range, 9 to 120 months), patients in EBV DNA-positive group displayed unfavorable progression-free survival (PFS) (P=0.012) and overall survival (OS) (P=0.004) than patients in EBV DNA-negative group. Multivariate Cox regression analysis revealed that EBV DNA-positivity was an independent risk factor for both PFS (HR, 2.04; 95% CI, 1.07 to 3.92; P=0.031) and OS (HR, 2.68; 95% CI, 1.20 to 6.00; P=0.016). Reduction in EBV copies was significantly associated with therapy-response. Circulating EBV DNA load in whole blood proved to be a significant predictor of prognosis in patients with MCL, which needs further validation in large-scale clinical studies.

The prognostic value of 25-hydroxy vitamin D deficiency and its interaction with c-Myc expression in diffuse large B cell lymphoma.

This study investigated the prognostic value of 25-hydroxy vitamin D (25-(OH)D) deficiency and the association between 25-(OH)D deficiency and c-Myc positivity in 208 newly diagnosed diffuse large B cell lymphoma (DLBCL) patients. 25-(OH)D deficiency was defined as serum 25-(OH)D level lower than 52.5 nmol/L. Using cutoff values of 40%, positive tumor cells for c-Myc expression was established. One hundred forty-two patients had 25-(OH)D deficiency and 70 had c-Myc positivity with a median follow-up of 29 months (range, 16 to 49 months) in this cohort. Multivariate Cox regression analysis showed that 25-(OH)D deficiency was an independent prognostic predictor for inferior progression-free survival (PFS) (P = 0.001) and overall survival (OS) (P = 0.006), and c-Myc positivity was an unfavorable prognostic factor for PFS (P = 0.004). In addition, c-Myc positivity was more frequent in patients with 25-(OH)D deficiency (P = 0.027). Moreover, we found that the presence of c-Myc positivity could aggravate the adverse effects of 25-(OH)D deficiency for PFS time (P = 0.0045). 25-(OH)D deficiency together with IPI (IPI-D) improved the prognostic capacity compared with only IPI in predicting the risk of DLBCL which was assessed by the calculation of receiver operator characteristic (ROC) curves and the areas under the curve (AUC). Noteworthy, c-Myc positivity combined with IPI-D was better than IPI-D in predicting PFS time. In summary, 25-(OH)D deficiency was a strong prognostic factor in DLBCL. Further multi-center prospective studies are needed to confirm the results and better understand the underlying mechanisms.

[Clinical Characteristics and Prognosis of 56 Lymphoma Patients with Mediastinal Masses].

OBJECTIVE: To analyze the incidence, clinical characteristics, treatment efficacy and prognosis of lymphoma patients with mediastinal masses. METHODS: The clinical data of 56 lymphoma patients with mediastinal masses treated in our hospital from February 2007 to January 2018 were collected, and the clinical characteristics, typing, staging, treatment efficacy as well as prognosis analyzed retrospectively. RESULTS: Among the 56 patients, male and female were 25 (45%) cases and 31(55%) cases, respectively. The median age was 31 (17-72) years old, out of them 48 (86%) patients aged below 40 years old. According to pathological classification, 9 (16.1%) cases were diffuse large B cell lymphoma, not otherwise specified (DLBCL-NOS), 26(46.4%) cases were primary mediastinal large B cell lymphoma (PMBCL), 17 (30.4%) cases were classical Hodgkin's lymphoma (cHL), and 4 (7.1%) cases were primary mediastinal grey zone lymphoma (MGZL). All the patients were treated with chemotherapy or immuno-chemotherapy.The median follow-up time was 54.5(11-149) months. In cHL group, 10 cases received involved field radiotherapy (IFRT), 10 (58.8%) cases achieved complete remission (CR), 2 (11.8%) cases achieved partial remisson (PR), and 5 (29.4%) patients experienced progressive disease (PD) , 6 patients with relapsed/refractory disease received autologous hematopoietic stem cell transplantation (auto-HSCT), the 1, 2, and 5 year overall survival (OS) rates of cHL group were 94.1%, 88.2%, and 67.2%, respectively. In MGZL group, 3 cases combined with IFRT, 1 case with auto-HSCT, 2 (50%) cases achieved CR, 2 (50%) cases experienced PD, the 1, 2, 5 year OS rates of MGZL group were 66.7%, 66.7%, and 33.3%, respectively. In PMBCL group, 8 cases combined with IFRT, 7 cases with auto-HSCT, 22 (84.0%) cases achieved CR, 2 (8.0%) cases achieved PR, 1 (4.0%) case was stable disease (SD) and 1 (4.0%) case experienced PD, the 1, 2 year OS rates of PMBCL group were both 100%, and 5 year OS rate was 95.7%. In DLBCL-NOS group, 3 cases combined with IFRT, 2 cases with auto-HSCT, 4 (44.5%) cases achieved CR, 2 (22.2%) cases achieved PR, 1 (11.1%) case was stable disease (SD) and 2 (22.2%) cases experienced PD, the 1 year OS rate of DLBCL-NOS cohort was 100%, 2, and 5 year OS rates were both 77.8%. There was a significant difference of OS rate among these 4 group (P＜0.05). In intra-group comparison, OS rate in PMBCL group were significantly longer than both MGZL group (P＜0.01) and cHL group(P＜0.05), and showed no significant difference from DLBCL-NOS group (P＞0.05). CONCLUSION: Mediastinal masses are characterized by unique clinical features due to their unique anatomical location. Several B cell originated lymphomas including cHL,MGZL,PMBCL and DLBCL-NOS, manifested primary or secondary mediastina involvement, tend to occur in young adults and have similar clinical features. However, their therapeutic response was significantly different. The prognosis of PMBCL is prior to MGZL and cHL.