Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

Blood ◽  
2008 ◽  
Vol 112 (8) ◽  
pp. 3082-3087 ◽  
Author(s):  
Suzanne O. Arulogun ◽  
H. Miles Prince ◽  
Jonathan Ng ◽  
Stephen Lade ◽  
Gail F. Ryan ◽  
...  
Keyword(s):  
De Novo ◽  
Cell Transformation ◽  
Early Stage ◽  
Large Cell ◽  
Tumor Stage ◽  
Advanced Stage ◽  
Long Term Outcomes ◽  
Stage Disease ◽  
Large Cell Transformation

Abstract Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and SS, was undertaken to study long-term outcomes and identify clinical predictors of outcome in patients with advanced-stage disease (ASD, n = 92) and large cell transformation (LCT, n = 22). Two-thirds of patients with ASD presented with de novo ASD. The median overall survival (OS) for ASD was 5 years with a 10-year predicted OS of 32%. Age at initial diagnosis (P = .01), tumor stage (P = .01), and clinical stage (P = .001) were found to be significant predictors of outcome. Patients who presented with de novo ASD demonstrated better outcomes that were not statistically significant than those with a prior diagnosis of early-stage MF (P = .25). Transformation developed in 22 of the 297 MF/SS patients (7.4%), with a transformation rate of only 1.4% in patients with early-stage disease, compared with stage IIB (27%) and stage IV (56%-67%) disease. The median OS from diagnosis of LCT was 2 years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis, and we demonstrate a much lower overall risk of LCT than previously reported.

Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sézary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

2010 ◽  
Vol 28 (31) ◽  
pp. 4730-4739 ◽  
Author(s):  
Nita Sally Agar ◽  
Emma Wedgeworth ◽  
Siobhan Crichton ◽  
Tracey J. Mitchell ◽  
Michael Cox ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
International Society ◽  
Cell Transformation ◽  
Early Stage ◽  
Large Cell ◽  
Early Stage Disease ◽  
Stage Disease ◽  
Cutaneous Lymphomas ◽  
Large Cell Transformation ◽  
Male Sex

Purpose We have analyzed the outcome of mycosis fungoides (MF) and Sézary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sézary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

New advances in the diagnosis and management of hepatocellular carcinoma

BMJ ◽  
2020 ◽  
pp. m3544 ◽  
Author(s):  
Ju Dong Yang ◽  
Julie K Heimbach
Keyword(s):  
Hepatocellular Carcinoma ◽  
Systemic Treatment ◽  
Early Stage ◽  
Treatment Options ◽  
Intermediate Stage ◽  
Phase Iii ◽  
Advanced Hepatocellular Carcinoma ◽  
Long Term Outcomes ◽  
Stage Disease

ABSTRACT Hepatocellular carcinoma is one of the leading causes of cancer related death in the world. Biannual surveillance for the disease in patients with cirrhosis and in high risk carriers of hepatitis B virus allows early stage cancer detection and treatment with good long term outcomes. Liver ultrasonography and serum α fetoprotein are the most commonly used surveillance tests. If suspicious results are found on the surveillance test, multiphasic computed tomography or magnetic resonance imaging should be undertaken to confirm the diagnosis of hepatocellular carcinoma. If radiologic tests show inconclusive results, liver biopsy or repeat imaging could be considered for confirmation of hepatocellular carcinoma. Management of the disease is complex. Patients should be evaluated by a multidisciplinary team, and the selection of treatment should consider factors such as tumor burden, severity of liver dysfunction, medical comorbidities, local expertise, and preference of patients. Early stage hepatocellular carcinoma is best managed by curative treatment, which includes resection, ablation, or transplantation. Patients with intermediate stage disease often receive locoregional treatment. Systemic treatment is reserved for patients with advanced disease. Several positive, phase III, randomized controlled trials have expanded the systemic treatment options for advanced hepatocellular carcinoma with promising long term outcomes, especially trials using combination treatments, which could also have eventual implications for the treatment of earlier stage disease.

Histologic Subtypes Do Not Confer Unique Outcomes in Early-Stage Lymphoma: Long-Term Follow-Up of SWOG 8736.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3263-3263 ◽  
Author(s):  
Catherine M. Spier ◽  
Michael LeBlanc ◽  
Ellen M. Chase ◽  
Richard I. Fisher ◽  
Thomas P. Miller
Keyword(s):  
T Cell ◽  
Early Stage ◽  
Large Cell ◽  
Advanced Stage ◽  
Histologic Type ◽  
Limited Disease ◽  
Histologic Subtype ◽  
Limited Stage ◽  
Stage Disease ◽  
Histologic Subtypes

Abstract Analysis of patients with advanced non-Hodgkin’s lymphoma (NHL) suggests unique clinical presentations and outcome according to histologic subtype. Little is known of outcome as it relates to histologic subytpe in patients with limited disease. In a previous report (NEJM1998;339:21–6), 401 patients with limited disease (Stage I or non-bulky II) intermediate or high grade NHL, enrolled in SWOG study 8736, were randomized to chemotherapy or chemotherapy plus radiotherapy. Results at 5 years showed superior survival and less toxicity for those treated with 3 cycles of CHOP chemotherapy plus radiotherapy. These 401 patients, with additional followup and reclassification of histology according to World Health Organization (WHO) criteria, form the basis of the current report. Of the 401 pts, 2 were excluded because of clinical findings and 34 patients were excluded for insufficient pathology material. 365 pts were evaluable. On the basis of hematoxylin-eosin and immunohistochemistry results, pt cases were classified as diffuse large B cell (174pts), follicular, Grade 3 (36), Burkitt-like (27), peripheral T cell, unspecified (9), anaplastic large T cell (4), morphologically consistent with mantle cell (4), morphologically consistent with MALToma (3), morphologically diffuse small cleaved cell (2), unclassifiable (1), suggesting anaplastic large cell CD30+ (1), diffuse large cell without immunohistochemical results (104). At 10 years, there was no significant difference in overall survival (OS) or failure free survival (FFS) between the 2 treatments (OS, p=0.48; FFS, p=0.77) (Blood2001;98:724a). When analyzed by histology, including T lineage, neither OS nor FFS were different for any histologic subytpe (OS, p=0.94; FFS, p=0.99). In addition, the estimated shapes of the survival curves were similar for all histologic subtypes among these 365 evaluable pts. On the basis of these findings it does not appear that either the WHO or the Working Formulation (WF) classification system is able to predict outcome for limited stage disease pts treated with CHOP +/− radiotherapy. Therefore, histologic type should not be a criterion for exclusion for limited stage studies of lymphoma. Further, it does not appear that any histologic subtype presenting as early stage disease needs CNS prophylaxis or a unique chemotherapy treatment strategy. There was only one central nervous system (CNS) relapse (in a patient with testis involvement), and none among the 27 Burkitt-like pts. The lack of an observed survival plateau in the first 10 years of followup for these pts is in contrast to advanced stage pts who achieve a plateau within 6 years. The biology of limited stage lymphoma may be fundamentally different from that of advanced stage disease and more significanct in determining outcome than histologic type.

scholarly journals Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model

2015 ◽  
Vol 33 (32) ◽  
pp. 3766-3773 ◽  
Author(s):  
Julia J. Scarisbrick ◽  
H. Miles Prince ◽  
Maarten H. Vermeer ◽  
Pietro Quaglino ◽  
Steven Horwitz ◽  
...  
Keyword(s):  
Lactate Dehydrogenase ◽  
Mycosis Fungoides ◽  
Prognostic Markers ◽  
Cell Transformation ◽  
Survival Rates ◽  
Prognostic Index ◽  
Stage Iv ◽  
Large Cell ◽  
Advanced Stage ◽  
Large Cell Transformation

Purpose Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers. Patients and Methods Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS). Results Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%). Conclusion To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.

scholarly journals Population-Based, Cause-Specific Risk of Non-Lymphoma Deaths Among 20,491 Adults with Classical Hodgkin Lymphoma (cHL) Treated with Initial Chemotherapy in the United States, 2000-2015

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4034-4034
Author(s):  
Graca M. Dores ◽  
Rochelle E. Curtis ◽  
Nicole H. Dalal ◽  
Martha S. Linet ◽  
Lindsay M. Morton
Keyword(s):  
Adverse Events ◽  
Early Stage ◽  
The United States ◽  
First Year ◽  
Advanced Stage ◽  
Treatment Approaches ◽  
The Past ◽  
Specific Risk ◽  
Stage Disease

Introduction: Advances in cHL treatment over the past 50 years have been fueled, in part, by recognition of long-term treatment-related complications that emerged after cure of cHL became a reality. While cHL-specific survival has continued to improve with time, premature death following diagnosis and treatment of cHL remains life-limiting. Most studies of cHL mortality have focused on long-term survivors and included patients treated with chemotherapy approaches and radiation techniques used in the past. Therefore, we sought to comprehensively quantify early and late cause-specific risks of death among U.S. adults with cHL treated with chemotherapy during a treatment era predominated by ABVD-based (doxorubicin, bleomycin, vinblastine, dacarbazine) chemotherapy. Methods: We used data from 17 cancer registry areas of the Surveillance, Epidemiology, and End Results Program to quantify risks of death among individuals diagnosed with early (I/II) or advanced (III/IV) stage cHL between ages 20-74 years and treated with initial chemotherapy during 2000-2015. We calculated standardized mortality ratios (SMRs) and 95% confidence intervals to compare cause-specific risk of death following cHL to that expected in the general U.S. population and estimated absolute excess risks (per 10,000 patient-years) to quantify disease-specific death burden. Results: Among 24,205 cHL patients, we assessed mortality among the 85% (n=20,491) treated with initial chemotherapy (37% of the 20,491 also received initial radiotherapy). With a mean follow-up of 6.7 years, we observed 3,230 deaths due to lymphoma (n=1,936), other neoplasms (n=273), noncancers (n=937), and unknown (n=84) causes. The risk of non-lymphoma deaths overall was significantly elevated 1.5- and 2.3-fold among adults diagnosed with early or advanced cHL, respectively, representing 20 (early stage) and 75 (advanced stage) excess deaths/10,000 patient-years. The most common causes of noncancer deaths were attributed to cardiovascular (n=284), respiratory (n=166), and infectious (n=119) diseases and accidents/falls/adverse events (n=121). The greatest increased risk of cardiovascular deaths was observed for heart disease <1-year after cHL which persisted 1-4 and >5 years after early and advanced stage cHL. Death from interstitial lung disease (ILD) significantly exceeded the expected rate by 14- and 24-fold in early and advanced stage cHL, respectively, most notably within the first year of diagnosis (SMRearly=90; SMRadvanced=128), with SMRs decreasing substantially after 1-year but remaining significantly increased in both stage groups. Patients were also prone to infection-related deaths irrespective of early (SMRearly=2.2) or advanced (SMRadvanced=3.9) cHL, both <1 or >1 year after diagnosis, with the greatest excesses occurring within the first year. Individuals with early (SMRearly=1.8) and advanced stage (SMRadvanced=4.2) disease had significantly elevated risks of death from adverse events (deaths coded as sequelae of drug, treatment, or other specified exposure), most notably <1 year after cHL diagnosis (SMRearly=7.5; SMRadvanced=17.5). Among non-lymphoma neoplasms, death from myelodysplastic syndromes/acute myeloid leukemia (MDS/AML) was significantly increased among early (SMRearly=5.2) and advanced stage (SMRadvanced=8.6) cHL. No MDS/AML deaths occurred within 1-year of cHL, but risks increased to >9-fold among both stage groups 1-4 years after diagnosis, and excesses persisted at >5-years among those with advanced stage cHL (SMRadvanced=9.1). Lung cancer accounted for the majority of solid tumor deaths among those with early (SMRearly=1.4) and advanced stage (SMRadvanced=1.9) cHL, with heightened risks beginning at >5 years among early stage cHL and at 1-4 years among advanced stage disease. Conclusion: Despite tailored treatment approaches in an ABVD-predominant era, risk of non-lymphoma deaths remains significantly elevated among cHL patients 20-74 years of age in the U.S., with early or advanced stage disease, and <1 or >1 year from diagnosis. Our data strongly support implementation of preventive and supportive measures following cHL diagnosis and continued refinement of treatment approaches to minimize premature deaths. Disclosures No relevant conflicts of interest to declare.

scholarly journals Early stage mycosis fungoides with focal CD30-positive large cell transformation

2013 ◽  
Vol 31 (2) ◽  
pp. 73-77 ◽  
Author(s):  
Po-Ju Lai ◽  
Yu-Ping Hsiao ◽  
Jeng-Dong Hsu ◽  
Shiow-Jiuan Wey
Keyword(s):  
Mycosis Fungoides ◽  
Cell Transformation ◽  
Early Stage ◽  
Large Cell ◽  
Large Cell Transformation

scholarly journals AB0546 5 YEARS FOLLOW-UP OF PSORIATIC ARTHRITIS PATIENTS TREATED ACCORDING TREAT-TO-TARGET STRATEGY. PRELIMINARY RESULTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1306.1-1306
Author(s):  
P. Tremaskina ◽  
E. Loginova ◽  
T. Korotaeva ◽  
S. Glukhova ◽  
A. Lila
Keyword(s):  
Psoriatic Arthritis ◽  
Disease Activity ◽  
Standard Care ◽  
Early Stage ◽  
Treat To Target ◽  
Moderate Activity ◽  
Long Term Outcomes ◽  
Mean Values

Background:The concept of treat to target (T2T) in psoriatic arthritis (PsA) has been established recently and already shown its benefits [1]. But the long-term outcomes of the T2T have not been studied yet.Objectives:To study 5 years (yrs) follow-up of PsA patients (pts) treated according to T2T strategy at the early stage.Methods:35 (M/F–17/18) PsA pts fulfilling CASPAR criteria, who were treated according to T2T strategy at the early stage (PsA duration≤2 yrs) within 24 months (mos) were analyzed. At the time of evaluation mean age is 42.7±11.2 yrs, median (Me) PsA duration 72 [60;95] mos, psoriasis duration 120 [88;180] mos. All pts underwent standard clinical examinations of PsA before started T2T therapy and at follow-up. Within 24 mos of T2T strategy all pts were taking Methotrexate (MTX) monotherapy in increasing dose up to 25 mg/wk and 18 out of 35 (51%) pts received MTX in combination with iTNF. When T2T study was stopped all pts were treated according to standard care with NSAIDs, bDMARDs, MTX, tsDMARDs based on PsA activity and physician decision. The number of pts achieved minimal disease activity (MDA, 5 of 7) and remission by DAPSA (≤4)/low disease activity (LDA)≤14) at the 24 mos of T2T strategy and at 5 yrs follow-up were calculated. The results are presented in the form of mean values, median, upper and lower quartiles.Results:Me duration of follow-up is 68 [53.5;81.5] mos. At 24 mos Me DAPSA 3.48 [0.45;21.76], remission by DAPSA (REM-DAPSA) were seen in 20 out of 35 (57%) pts, LDA-DAPSA in 4 (12%) pts, moderate activity (MoA) by DAPSA in 6 (17%) pts and high disease activity by DAPSA (HDA-DAPSA) in 5 (14%) pts. MDA was noted in 21 out of 35 (60%) pts. At 5 yrs Me DAPSA 7.4 [2.22;13.87], REM-DAPSA was noted in 12 (34%) pts, LDA-DAPSA in 14 (40%), MoA-DAPSA in 5 (14%), HDA-DAPSA in 4 (12%) pts. MDA was observed in 17 of 35 pts (49%). Among 20 pts who had REM-DAPSA at 24 mos only 6 pts (30%) remained in remission at 5 yrs follow-up and 12 out of 21 pts (57.14%) remained in MDA status.Conclusion:In early PsA pts remission and MDA are achievable goal of T2T strategy. But most pts lost remission/MDA after this strategy was changed to a standard care, despite being in remission/MDA status before change of therapy. Further investigations of the long-term outcomes of T2T strategy in PsA, including radiographic outcomes are needed.References:[1]Coates LC, Moverley AR, McParland L, et al. Lancet 2015; 386: 2489–98.Disclosure of Interests:None declared.

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