-
Cancer Science Dec 2020Adult T-cell leukemia/lymphoma (ATL) is a mature T-cell neoplasm and is classified into four subtypes (acute, lymphoma, chronic, and smoldering) according to the... (Observational Study)
Observational Study
Adult T-cell leukemia/lymphoma (ATL) is a mature T-cell neoplasm and is classified into four subtypes (acute, lymphoma, chronic, and smoldering) according to the Shimoyama classification, established in 1991 through several nationwide surveys based on the clinical diversity of patients diagnosed in 1983-1987 in Japan. Thereafter, no such studies have been conducted. Recently, we conducted a nationwide hospital survey using the method of the 1980s studies, collected baseline data on 996 ATL patients diagnosed in 2010-2011 from 126 hospitals, and reported their unique epidemiological characteristics. Here, we report the follow-up results of registered ATL patients with the goal of evaluating current prognoses and treatment modalities as of 2016-2017. Of 770 evaluable patients, 391 (50.8%) had acute-type, 192 (24.9%) had lymphoma-type, 106 (13.8%) had chronic-type, and 81 (10.5%) had smoldering-type ATL. The initial therapy regimens used for acute/lymphoma-type ATL were vincristine, cyclophosphamide, doxorubicin and prednisone, followed by doxorubicin, ranimustine, and prednisone and then by vindesine, etoposide, carboplatin, and prednisone (VCAP-AMP-VECP)-like in 38.5/41.7% and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)-like in 14.6/13.7% of patients. Allogeneic hematopoietic stem cell transplantation was used to treat 15.9/10.4% of acute/lymphoma-type ATL patients. The 4-year survival rates (the median survival time, days) for acute-, lymphoma-, unfavorable chronic-, favorable chronic-, and smoldering-type ATL were 16.8% (252), 19.6% (305), 26.6% (572), 62.1% (1937), and 59.8% (1851), respectively. The 4-year survival rates for acute- and lymphoma-type ATL improved compared with those reported in 1991, but those for chronic- and smoldering-type ATL were not. Further efforts are warranted to develop more efficient therapeutic strategies to improve the prognosis of ATL in Japan.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Cause of Death; Cyclophosphamide; Doxorubicin; Female; Health Care Surveys; Hematopoietic Stem Cell Transplantation; Hospitals; Humans; Japan; Leukemia-Lymphoma, Adult T-Cell; Male; Middle Aged; Nitrosourea Compounds; Prednisone; Prognosis; Retrospective Studies; Survival Rate; Vincristine; Vindesine
PubMed: 32976684
DOI: 10.1111/cas.14658 -
Clinical Lymphoma, Myeloma & Leukemia Oct 2020Despite its low frequency in all variants of diffuse large B-cell lymphoma (DLBCL), CD5 DLBCL has gradually gained the attention it deserves, the result of its poorer... (Review)
Review
Despite its low frequency in all variants of diffuse large B-cell lymphoma (DLBCL), CD5 DLBCL has gradually gained the attention it deserves, the result of its poorer outcomes compared to DLBCL without the CD5 signature. CD5 DLBCL is classified as activated B-cell-like (ABC)/non-germinal-center B-cell-like (GCB) DLBCL with elusive genetic features, and patients are frequently characterized as being older and female, and as having Eastern Cooperative Oncology Group performance status > 1, high International Prognostic Index score, tendency to develop B symptoms, and advanced-stage disease with high central nervous system relapse and bone marrow involvement rate. The mechanism underlying the poor prognosis in CD5 DLBCL has not been fully explored, and we summarize the reported potential mechanisms, including CD5-mediated B-cell receptor (BCR)-dependent and -independent pathways. The former involves the inhibition of BCR signaling, and the latter involves the BCR-independent overexpression of interleukin 10, Bcl-2 (antiapoptotic B-cell leukemia/lymphoma 2), cyclin D2, and CXCR4 (C-X-C motif chemokine receptor 4). The efficacy of traditional regimen R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) is currently not satisfied in CD5 DLBCL. Therapies of larger doses, such as R-DA-EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin plus rituximab), R-ACVBP (rituximab plus doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone), R-DA-EPOCH plus central nervous system prophylaxis, can improve the overall survival in CD5 DLBCL patients, while allogeneic hematopoietic stem-cell transplantation still remains controversial as a salvage treatment. In addition, some novel drugs, such as lenalidomide, CXCR4 antagonists, Bruton tyrosine kinase inhibitors, Bcl-2 inhibitors, and immunotherapy, have been reported to have encouraging results and may improve the outcomes of these patients. In the present review, we comprehensively summarize the biology, mechanism, and treatment of CD5 DLBCL.
Topics: CD5 Antigens; Female; Humans; Lymphoma, Large B-Cell, Diffuse; Male
PubMed: 32694049
DOI: 10.1016/j.clml.2020.05.003 -
OncoTargets and Therapy 2020Primary pulmonary diffuse large B cell lymphoma (PPDLBCL) is extremely rare, with fewer than 40 cases reported to date and a lack of systemic analysis. Herein, we...
Primary pulmonary diffuse large B cell lymphoma (PPDLBCL) is extremely rare, with fewer than 40 cases reported to date and a lack of systemic analysis. Herein, we present a case of PPDLBCL mimicking metastasis in a heavily treated patient with breast cancer. To our knowledge, this is the first reported case of PPDLBCL in a patient with breast cancer. A 66-year-old Chinese female diagnosed with breast cancer 7.5 years previously and multiple bone metastases 31 months later presented with a new-onset subpleural nodule in the inferior lobe of left lung detected by routine follow-up in November 2017. A 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography scan showed that the pulmonary nodule was hypermetabolic with a maximum standard uptake value of 14.9, consistent with lung metastasis in view of her history of breast cancer and multiple bone involvement. Surprisingly, pathologic investigation revealed primary lung DLBCL, staged IEA. Systemic chemotherapy with R-CDOP (rituximab, cyclophosphamide, vindesine, doxorubicin liposome, and prednisone) achieved complete remission with mild side effects. At the latest follow-up in August 2019, the patient had disease-free survival of 21 months. The findings from this case indicate that primary pulmonary lymphoma should be included in the differential diagnostic checklist of pulmonary occupancy, even in solid tumor patients treated with multiple modalities. When a newly developed lung nodule is identified in such patients, clinicians should not take for granted that it is lung metastasis. Pathology results are a prerequisite for making a correct diagnosis, choosing appropriate treatment, and improving patient prognosis.
PubMed: 32606794
DOI: 10.2147/OTT.S251344 -
Zhonghua Xue Ye Xue Za Zhi = Zhonghua... Mar 2020To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma (ALCL) . 40 ALCL cases treated in the First Affiliated...
To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma (ALCL) . 40 ALCL cases treated in the First Affiliated Hospital of Zhejiang University from January 2013 to December 2018 were retrospectively analyzed. ① With a median age of 41 (14-67) years, there were 29 males and 11 females, 36 patients (90.0%) had Ann Arbor stage Ⅲ-Ⅳ tumors, 23 patients (57.5%) were in high-intermediate or high international prognostic index (IPI) risk group. 25 patients (62.5%) had B symptoms, such as fever, emaciation and night sweat.38 patients (95.0%) had extranodal invasion, 25 patients (62.5%) had higher LDH level, and 25 patients (62.5%) had high expression of Ki-67 (80% or more) . With 22 ALK(+) patients (55.0%) and 18 ALK(-) patients (45.0%) , there was a significantly difference in the median age of the two groups [29 (14-67) years old 51.5 (19-67) years old, =0.003]. ② All patients received chemotherapy, 18 cases were treated with CHOP (cyclophosphamide, doxorubicin, vindesine, prednisone) , 12 cases with ECHOP (cyclophosphamide, doxorubicin, vindesine, prednisone, etoposide) , 10 cases with other treatments and 26 patients (65.0%) obtained complete remission (CR) . ALK(-) (=0.029, =13.458) and Ki-67 expression of 80% or more (=0.04, =14.453) were independent factors of CR rate, the CR rate of ECHOP chemotherapy was higher than CHOP chemotherapy (=0.026) . ③ LDH level, IPI score, ALK expression and chemotherapy regimen had significantly effect on progression free survival (PFS) and overall survival (OS) (<0.05) . The study shows that primary systemic ALCL usually occurs in males, the average age of ALK(+) patients were younger than ALK(-) patients. Most patients are in stage Ⅲ-Ⅳ with extranodal invasion, more than half of the patients have B symptoms, elevated LDH, and high expression of Ki-67. The expression level of Ki-67, ALK expression, and chemotherapy regimen have prognostic value for CR rate, the LDH level, IPI score, ALK expression and chemotherapy regimen for PFS and OS. ECHOP is a better choice with improved prognosis.
Topics: Adolescent; Adult; Aged; Anaplastic Lymphoma Kinase; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Doxorubicin; Female; Humans; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Large-Cell, Anaplastic; Male; Middle Aged; Prednisone; Prognosis; Receptor Protein-Tyrosine Kinases; Retrospective Studies; Vincristine; Young Adult
PubMed: 32311892
DOI: 10.3760/cma.j.issn.0253-2727.2020.03.007 -
Medicine Feb 2020The specific pathogenesis of the diffuse large B-cell lymphoma(DLBCL)is still indefinite and argumentative. It is known that DLBCL is the most common type of...
RATIONALE
The specific pathogenesis of the diffuse large B-cell lymphoma(DLBCL)is still indefinite and argumentative. It is known that DLBCL is the most common type of non-Hodgkin's lymphomas (NHL). A lot of cases of DLBCL such as primary gastric diffuse large B-cell lymphoma(PG-DLBCL) are reported. However, primary intestinal diffuse large B-cell lymphoma(PI-DLBCL) is unusual.
PATIENT CONCERNS
We present a case of a 57-year-old male diagnosed in the Gastroenterology Department, which presented a bleeding duodenal ulcer with irregular borders.
DIAGNOSES
The immunohistochemical staining showed: CD20(+++), CD10(+) and Ki-67>40%.
INTERVENTIONS
The patient was successfully treated by Poly-chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vindesine and prednisolone).
OUTCOMES
After 6 courses of chemotherapy treatment, the duodenal ulcer was completely healed by reviewing the UGIE.
LESSONS
Our report might give further strength to avoiding the erroneous and missed diagnosis for PI-DLBCL which is different from common duodenal ulcer.
Topics: Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Diagnosis, Differential; Doxorubicin; Duodenal Ulcer; Humans; Immunohistochemistry; Intestinal Neoplasms; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prednisone; Rituximab; Vincristine
PubMed: 32028388
DOI: 10.1097/MD.0000000000018590 -
British Journal of Haematology Nov 2019Patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL) after, or ineligible for, autologous stem cell transplantation (ASCT) have a dismal prognosis.... (Clinical Trial)
Clinical Trial
Rituximab-PECC induction followed by Y-ibritumomab tiuxetan consolidation in relapsed or refractory DLBCL patients who are ineligible for or have failed ASCT: results from a phase II HOVON study.
Patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL) after, or ineligible for, autologous stem cell transplantation (ASCT) have a dismal prognosis. This phase II study evaluated treatment with R-PECC (rituximab, prednisolone, etoposide, chlorambucil, lomustine), every 28 days for 4 cycles in 62 patients, followed by radio-immunotherapy consolidation with Y-ibritumomab tiuxetan in responsive patients. Primary endpoints were failure-free survival (FFS) and incidence of grade ≥3 adverse events from start of Y-ibritumomab tiuxetan. The overall response rate after R-PECC was 50%. Twenty-nine of 31 responsive patients proceeded to Y-ibritumomab tiuxetan. Five out of 15 partial remission patients converted to complete remission after Y-ibritumomab tiuxetan. One-year FFS and overall survival (OS) from start of Y-ibritumomab tiuxetan was 52% (95% confidence interval [CI], 33-68%) and 62% (95% CI, 42-77%), respectively. One-year FFS and OS from start of R-PECC was 28% (95% CI, 17-39%) and 49% (95% CI, 36-61%), respectively. Toxicities of R-PECC and Y-ibritumomab tiuxetan were mainly haematological. In conclusion, for relapsed DLBCL patients the largely oral R-PECC regimen achieves promising response rates, combined with an acceptable safety profile. Consolidation with Y-ibritumomab tiuxetan resulted in long-term response durations in approximately one third of the patients that received it.
Topics: Aged; Aged, 80 and over; Antibodies, Monoclonal; Antineoplastic Combined Chemotherapy Protocols; Autografts; Carmustine; Cyclophosphamide; Disease-Free Survival; Female; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prednisone; Rituximab; Stem Cell Transplantation; Survival Rate; Vindesine
PubMed: 31290569
DOI: 10.1111/bjh.16087 -
Journal of Nuclear Medicine : Official... Jan 2020We assessed the predictive value of new radiomic features characterizing lesion dissemination in baseline F-FDG PET and tested whether combining them with baseline...
We assessed the predictive value of new radiomic features characterizing lesion dissemination in baseline F-FDG PET and tested whether combining them with baseline metabolic tumor volume (MTV) could improve prediction of progression-free survival (PFS) and overall survival (OS) in diffuse large B-cell lymphoma (DLBCL) patients. From the LNH073B trial (NCT00498043), patients with advanced-stage DLCBL and F-FDG PET/CT images available for review were selected. MTV and several radiomic features, including the distance between the 2 lesions that were farthest apart (Dmax), were calculated. Receiver-operating-characteristic analysis was used to determine the optimal cutoff for quantitative variables, and Kaplan-Meier survival analyses were performed. With a median age of 46 y, 95 patients were enrolled, half of them treated with R-CHOP biweekly (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) and the other half with R-ACVBP (rituximab, doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone), with no significant impact on outcome. Median MTV and Dmax were 375 cm and 45 cm, respectively. The median follow-up was 44 mo. High MTV and Dmax were adverse factors for PFS ( = 0.027 and = 0.0003, respectively) and for OS ( = 0.0007 and = 0.0095, respectively). In multivariate analysis, only Dmax was significantly associated with PFS ( = 0.0014) whereas both factors remained significant for OS ( = 0.037 and = 0.0029, respectively). Combining MTV (>384 cm) and Dmax (>58 cm) yielded 3 risk groups for PFS ( = 0.0003) and OS ( = 0.0011): high with 2 adverse factors (4-y PFS and OS of 50% and 53%, respectively, = 18), low with no adverse factor (94% and 97%, = 36), and an intermediate category with 1 adverse factor (73% and 88%, = 41). Combining MTV with a parameter reflecting the tumor burden dissemination further improves DLBCL patient risk stratification at staging.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Cyclophosphamide; Disease-Free Survival; Doxorubicin; Female; Fluorodeoxyglucose F18; Humans; Kaplan-Meier Estimate; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Multivariate Analysis; Neoplasm Metastasis; Neoplasm Staging; Positron-Emission Tomography; Prednisone; ROC Curve; Risk Assessment; Rituximab; Treatment Outcome; Vincristine; Vindesine; Young Adult
PubMed: 31201248
DOI: 10.2967/jnumed.119.229450 -
British Journal of Haematology Aug 2019JCOG9801, a randomized phase III trial, reported that vincristine, cyclophosphamide, doxorubicin and prednisone (VCAP); doxorubicin, ranimustine and prednisone (AMP);... (Randomized Controlled Trial)
Randomized Controlled Trial
Possibility of a risk-adapted treatment strategy for untreated aggressive adult T-cell leukaemia-lymphoma (ATL) based on the ATL prognostic index: a supplementary analysis of the JCOG9801.
JCOG9801, a randomized phase III trial, reported that vincristine, cyclophosphamide, doxorubicin and prednisone (VCAP); doxorubicin, ranimustine and prednisone (AMP); and vindesine, etoposide, carboplatin and prednisone (VECP) (VCAP-AMP-VECP; mLSG15) showed superior clinical outcomes when compared to cyclophosphamide, doxorubicin, vincristine and prednisone every 2 weeks (CHOP-14; mLSG19) in patients with untreated aggressive adult T-cell leukaemia-lymphoma (ATL). To identify patients who require VCAP-AMP-VECP, we conducted a supplementary analysis of JCOG9801. Overall, 105 patients were included and categorized into low- (n = 44), intermediate- (n = 54) and high-risk (n = 7) groups according to the age-adjusted ATL prognostic index (ATL-PI). We excluded the high-risk group due to small numbers of patients. VCAP-AMP-VECP did not show any superior trend for overall survival (OS) in the low-risk group (hazard ratio: 1·04; 95% confidence interval: 0·54-2·04). Better OS was observed in the intermediate-risk group treated with VCAP-AMP-VECP (hazard ratio: 0·65; 95% confidence interval: 0·36-1·19). In the intermediate-risk group, the VCAP-AMP-VECP arm showed higher complete response rates than the CHOP-14 arm (44·0% vs. 13·8%). The VCAP-AMP-VECP arm in both risk groups exhibited grade 4 thrombocytopenia, while grade 4 neutropenia was only observed in the intermediate-risk group. VCAP-AMP-VECP remains suitable for the intermediate-risk group, whereas its benefits appear modest in the low-risk group.
Topics: Adolescent; Adult; Aged; Female; Humans; Leukemia-Lymphoma, Adult T-Cell; Male; Middle Aged; Prognosis; Prospective Studies; Young Adult
PubMed: 31099033
DOI: 10.1111/bjh.15950