scholarly journals Time from Diagnosis to Initiation of Curative Chemotherapy Affects Outcomes in DLBCL - Data from a Large Asian Cohort

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1871-1871
Author(s):  
Colin Phipps ◽  
Yuh Shan Lee ◽  
Chandramouli Nagarajan ◽  
Yunxin Chen ◽  
Allan ZK Goh ◽  
...  
Keyword(s):  
Cox Regression ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
Continuous Variable ◽  
Disease Stage ◽  
Advanced Stage ◽  
Early Stage Disease ◽  
Stage Disease ◽  
Stage 1 ◽  
The Impact

Abstract Diffuse large B-cell lymphoma (DLBCL) is a high-grade lymphoma that requires treatment. It remains unclear how promptly curative anthracycline-based immunochemotherapy should be started and the prognostic effects time from diagnosis to treatment (TDT) has. We retrospectively analyzed the impact of TDT on progression-free survival (PFS) and overall survival (OS) using patient characteristics available at diagnosis. From the databases of two large lymphoma treatment centers in Singapore, we included 581 DLBCL patients sequentially diagnosed and treated with R-CHOP (or R-EPOCH) between 2002 and 2014. All transformed lymphomas and retroviral positive cases were excluded. Patients with TDT > 7 weeks were also excluded as only a few (N=17) in our dataset were, and in real world practice would be, treated beyond this time. The effect of TDT on PFS and OS was examined in Cox regression models which included other known prognostic variables of age, extranodal (EN) sites, lactate dehydrogenase (LDH), performance status, stage, international prognostic index (IPI) risk and cell-of-origin (COO) based on Hans' algorithm. TDT was treated as a continuous variable in per week time units. As well as in a binary normal vs. high model, LDH was analyzed as a continuous variable and then categorically following the NCCN-IPI concept of a normalized LDH to provide more clinical relevance. The median age was 60 years (range, 15-88) and median follow up in surviving patients 47 months. The number of patients with TDT in week 1 = 97, week 2 = 199, week 3 = 126, week 4 = 81, week 5 = 41, week 6 = 28, week 7 = 9. The median TDT was 14 days (range, 4-49) and was significantly longer in patients with normal LDH level, stage 1-2 disease, EN ≤ 1 site, and low IPI. In univariate analysis, longer TDT was associated with poorer PFS and OS in the high LDH and stage 3-4 groups. Cox regression analysis for PFS showed that poorer performance state of ECOG ≥ 2 (P=0.029), stage 3-4 disease (P<0.001), increased LDH 1-2 fold (P=0.01) and ≥ 2-fold (P <0.001), and longer TDT (P=0.008; HR 1.144, 95% CI 1.036 - 1.263) were predictive. For OS, stage 3-4 disease (P=0.019), increase in LDH 1-2 fold (P=0.005) and ≥ 2-fold (P <0.001), and longer TDT (P=0.01, HR 1.154; 95% CI 1.035 - 1.287) were significantly associated. Considering the statistically significant interactions between LDH and disease stage and TDT, we performed Cox regression separately for patients with high (>normal) and normal LDH, and for advanced stage (3-4) and early stage (1-2) disease. We found that TDT remained a significant predictor of PFS and OS in patients with advanced stage but not early stage disease and in high LDH but not normal level LDH patients. The analysis demonstrated that delays in starting curative immunochemotherapy have detrimental effects on PFS and OS in patients with high LDH and advanced stage disease. The benefit of starting treatment earlier in DLBCL patients with higher tumor bulk, while intuitive, has not been previously demonstrated. Our findings suggest that treatment delays, whenever possible, should be minimized. Disclosures Hwang: Janssen: Honoraria, Other: Travel support; MSD: Honoraria, Other: Travel support; Celgene: Honoraria, Other: Travel support; Roche: Honoraria, Other: Travel support; Sanofi: Honoraria, Other: Travel support; Pfizer: Honoraria, Other: Travel support; BMS: Honoraria, Other: Travel support; Novartis: Honoraria, Other: Travel support.

Evaluation of treatment outcome in 175 patients with Hodgkin lymphoma aged 60 years or over: the SHIELD study

Blood ◽  
2012 ◽  
Vol 119 (25) ◽  
pp. 6005-6015 ◽  
Author(s):  
Stephen J. Proctor ◽  
Jennifer Wilkinson ◽  
Gail Jones ◽  
Gillian C. Watson ◽  
Helen H. Lucraft ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Cox Regression ◽  
Early Stage ◽  
Univariate Analysis ◽  
Central Element ◽  
Disease Stage ◽  
Advanced Stage ◽  
Early Stage Disease ◽  
Large Patient ◽  
Stage Disease

Abstract The SHIELD program for Hodgkin lymphoma in patients 60 years of age or older, prospectively evaluated clinical features and outcome in a large patient cohort (n = 175). The central element was a phase 2 study of VEPEMB chemotherapy (n = 103, median age 73 years) incorporating comorbidity assessment. A total of 72 other patients were treated off-study but registered prospectively and treated concurrently with: ABVD (n = 35); CLVPP (n = 19), or other (n = 18). Of VEPEMB patients, 31 had early-stage disease (stage 1A/2A) and received VEPEMB 3 times plus radiotherapy. Median follow-up was 36 months. Complete remission (CR) rate (intention-to-treat) was 74% and 3-year overall survival (OS) and progression-free survival (PFS) were 81% and 74%, respectively. A total of 72 patients had advanced-stage disease (stage 1B/2B/3 or 4) and received VEPEMB 6 times. CR rate was 61% with 3-year OS and PFS of 66% and 58%, respectively. Of patients achieving CR, 13% with early-stage and 5% with advanced-stage disease progressed. Overall treatment-related mortality was 7%. In patients treated with curative intent with VEPEMB, ABVD, and CLVPP (n = 157), CR linked to several factors in univariate analysis. In a Cox regression model only, obtaining CR remained significant for OS and CR plus comorbidity and age for PFS. RS-EBV status had no significant effect on outcome.

scholarly journals A Retrospective Analysis of Outcomes of Diffuse Large B Cell Lymphoma (DLBCL) in the Elder Patients in China

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5410-5410
Author(s):  
Peng Liu ◽  
Ying Han ◽  
Jianliang Yang ◽  
Xiaohui He ◽  
Changgong Zhang ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
Disease Stage ◽  
Early Stage Disease ◽  
Large B Cell Lymphoma ◽  
Elder Patients ◽  
Stage Disease ◽  
Ann Arbor ◽  
Advanced Disease Stage

Abstract Background: Elderly patients with diffuse large B-cell lymphoma (DLBCL) have a worse prognosis compared to the younger population, since older age is associated with comorbidity and suboptimal performance status leading to intolerance of chemo-immunotherapy. The outcome of DLBCL in the older patients (> 60 years) was well described in clinical trials with reported 5-year overall survival (OS) of 50-80% (Coiffier et al., N Engl J Med 2002). Since this group is often precluded from clinical trials and population-based studies are limited, optimal treatment strategy for the old patients with DLBCL remains controversial. Here, we describe a Chinese real-world experience of management of elderly DLBCL patients treated at National Cancer Hospital, China. Methods: This is a single-center, retrospective analysis of consecutive DLBCL patients aged ≥60 who planned to receive chemotherapy +/- rituximab. The standard regimens included 3-4 cycles (early stage disease) or 6 cycles (advanced) of R-CHOP like regimens (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) followed by two rituximab doses in fit patients; R-miniCHOP for unfit and R-CE(etoposide)OP for frail elder patients. Patient data including baseline characteristics, histology, clinical parameters, and treatment outcomes were extracted from hospital medical records. The primary endpoint was progression-free survival (PFS); secondary endpoint was OS. Statistical analyses included descriptive statistics and Kaplan Meier estimates. Results: From June 2006 to December 2012, 349 patients aged ≥60 years were included, in which 204 patients were aged <70 years (Table 1). 326 patients received chemotherapy or chemo-immunotherapy with rituximab. Median follow up was 82 months. Five year PFS and OS of the elder patients were 45.8% and 51.9%, respectively. Significant difference was seen between patients < 70 years and those ≥70 years in terms of PFS (51.0% vs 38.6%, p=0.030) and OS (58.3% vs 42.8%, p=0.007) (Figure 1). Patients with early-stage disease (Ann Arbor StageⅠ/Ⅱ) had better 5-year PFS (60.1% vs 23.5%, p<0.001) and OS (65.3% vs 30.9%, p<0.001) than patients with advanced disease stage (Ann Arbor Stage III/IV) (Figure2). In addition, regimen including rituximab significantly improved the survival than chemotherapy alone (5-year PFS: 37.3% vs 64.0%, p<0.001; 5-year OS: 44.5% vs 69.3%, p<0.001), especially in patients ≥70 years, which almost doubled 5-year PFS and OS (5-year PFS: 25.4% vs 50.7%, p<0.001; 5-year OS: 28.8% vs 56.0%, p<0.001) (Figure3). Conclusions: Elder age (≥70 years) and advanced disease stage (Ann Arbor Stage III/IV) are associated with poor PFS and OS in Chinese elder DLBCL patients. The addition of rituximab significantly improves the survival compared to chemotherapy alone, especially in patients aged ≥70 years. These findings underscore the importance of personalized evaluation and treatment in elder patients with DLBCL. Disclosures No relevant conflicts of interest to declare.

Management of Melanoma during Pregnancy: A Case Series of 11 Women Treated at NYU Langone Health

Oncology ◽  
2020 ◽  
Vol 98 (12) ◽  
pp. 847-852
Author(s):  
Amelia E. Sawyers ◽  
Anna C. Pavlick ◽  
Jeffrey S. Weber ◽  
Iman Osman ◽  
Jennifer A. Stein
Keyword(s):  
Early Stage ◽  
Case Series ◽  
Advanced Stage ◽  
Early Stage Disease ◽  
Melanoma Diagnosis ◽  
Stage Disease ◽  
Treatment Plans ◽  
Melanoma Patients ◽  
The Impact

<b><i>Objectives:</i></b> Melanoma is one of the most common malignancies diagnosed during pregnancy. This study examined the impact of pregnancy on management decisions of melanoma patients treated at NYU Langone Health (NYULH). <b><i>Methods:</i></b> We analyzed data for patients who were pregnant at initial or recurrent melanoma diagnosis at NYULH from 2012 to 2019 with prospective protocol-driven follow-up. <b><i>Results:</i></b> Of the 900 female patients accrued during this period, 11 women in the childbearing range were pregnant at melanoma diagnosis. Six patients presented with early (stage 0 or I) disease and five with advanced (stage III or IV) melanoma. Women with early stage disease had normal deliveries and minimal changes to their treatment timeline and regimen. However, patients with more advanced stage disease opted for either termination of the pregnancy or early delivery and altered treatment timelines because of pregnancy. <b><i>Conclusion:</i></b> Both melanoma stage and gestational age at diagnosis contribute to the differences in the therapeutic management of melanoma in pregnant women. Given the complexity and variety of each case of melanoma during pregnancy, informed discussion between patients and physicians allows for individualized treatment plans that address each patient’s unique situation.

scholarly journals Health disparities and impact on outcomes in children with primary central nervous system solid tumors

2016 ◽  
Vol 18 (5) ◽  
pp. 585-593 ◽  
Author(s):  
Mary T. Austin ◽  
Emma Hamilton ◽  
Denna Zebda ◽  
Hoang Nguyen ◽  
Jan M. Eberth ◽  
...  
Keyword(s):  
Health Disparities ◽  
Solid Tumors ◽  
Survival Probability ◽  
Cox Regression ◽  
Disease Stage ◽  
Advanced Stage ◽  
Stage Disease ◽  
Hispanic Patients ◽  
The Impact ◽  
Racial Ethnic

OBJECTIVE Health disparities in access to care, early detection, and survival exist among adult patients with cancer. However, there have been few reports assessing how health disparities impact pediatric patients with malignancies. The objective in this study was to examine the impact of racial/ethnic and social factors on disease presentation and outcome for children with primary CNS solid tumors. METHODS The authors examined all children (age ≤ 18 years) in whom CNS solid tumors were diagnosed and who were enrolled in the Texas Cancer Registry between 1995 and 2009 (n = 2421). Geocoded information was used to calculate the driving distance between a patient's home and the nearest pediatric cancer treatment center. Socioeconomic status (SES) was determined using the Agency for Healthcare Research and Quality formula and 2007–2011 US Census block group data. Logistic regression was used to determine factors associated with advanced-stage disease. Survival probability and hazard ratios were calculated using life table methods and Cox regression. RESULTS Children with advanced-stage CNS solid tumors were more likely to be < 1 year old, Hispanic, and in the lowest SES quartile (all p < 0.05). The adjusted odds ratios of presenting with advanced-stage disease were higher in children < 1 year old compared with children > 10 years old (OR 1.71, 95% CI 1.06–2.75), and in Hispanic patients compared with non-Hispanic white patients (OR 1.56, 95% CI 1.19–2.04). Distance to treatment and SES did not impact disease stage at presentation in the adjusted analysis. Furthermore, 1- and 5-year survival probability were worst in children 1–10 years old, Hispanic patients, non-Hispanic black patients, and those in the lowest SES quartile (p < 0.05). In the adjusted survival model, only advanced disease and malignant behavior were predictive of mortality. CONCLUSIONS Racial/ethnic disparities are associated with advanced-stage disease presentation for children with CNS solid tumors. Disease stage at presentation and tumor behavior are the most important predictors of survival.

Lymphocyte-predominant Hodgkin lymphoma: what is the optimal treatment?

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 406-413 ◽  
Author(s):  
Michelle Fanale
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Radiation Treatment ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
B Cell Lymphomas ◽  
Early Stage Disease ◽  
Stage Disease ◽  
Relapsed Disease

AbstractNodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a unique diagnostic entity, with only ∼ 500 new cases in the United States per year with a similar infrequent incidence worldwide. NLPHL also has distinctive pathobiology and clinical characteristics compared with the more common classical Hodgkin lymphoma (cHL), including CD20 positivity of the pathognomic lymphocytic and histiocytic cells and an overall more indolent course with a higher likelihood of delayed relapses. Given the limited numbers of prospective NLPHL-focused trials, management algorithms historically have typically been centered on retrospective data with guidelines often adopted from cHL and indolent B-cell lymphoma treatment approaches. Key recent publications have delineated that NLPHL has a higher level of pathological overlap with cHL and the aggressive B-cell lymphomas than with indolent B-cell lymphomas. Over the past decade, there has been a series of NLPHL publications that evaluated the role of rituximab in the frontline and relapsed setting, described the relative incidence of transformation to aggressive B-cell lymphomas, weighed the benefit of addition of chemotherapy to radiation treatment for patients with early-stage disease, considered what should be the preferred chemotherapy regimen for advanced-stage disease, and even assessed the potential role of autologous stem cell transplantation for the management of relapsed disease. General themes within the consensus guidelines include the role for radiation treatment as a monotherapy for early-stage disease, the value of large B-cell lymphoma–directed regimens for transformed disease, the utility of rituximab for treatment of relapsed disease, and, in the pediatric setting, the role of surgical management alone for patients with early-stage disease.

scholarly journals Tumor Characteristics and Treatment Outcomes of Older Women with Breast Cancer in Jordan

2019 ◽  
Author(s):  
Hikmat Abdel-Razeq ◽  
Fadwa Abdel Rahman ◽  
Hanan Al-Masri ◽  
Hazem Abdulelah ◽  
Mahmoud Abu Nasser ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Treatment Outcomes ◽  
Metastatic Disease ◽  
Cox Regression ◽  
Early Stage ◽  
Poor Performance Status ◽  
Tumor Characteristics ◽  
Early Stage Disease ◽  
Node Positive ◽  
Stage Disease

Abstract Background : Less than 10% of newly diagnosed breast cancer in our region are diagnosed in women 70 years or older. Treatment plans of such patients is less clear and have poor outcomes. In this paper, we describe clinical presentation, tumor characteristics and treatment outcomes in such patients. Methods : Consecutive patients aged 65 years or older with pathologically-confirmed diagnosis of breast cancer were included. Medical records and hospital databases were searched for patients’ characteristics and treatment outcomes. Results : A total of 553 patients, median age 70 (range: 65-91) years, were included. On presentation, 114 (20.6%) patients had metastatic disease and was mostly visceral (81; 71.1%). Patients with non-metastatic disease had poor pathological features including node-positive in 244 (55.6%), GIII in 170 (38.7%) and lymphovascular invasion in 173 (39.4%). Patients were treated less aggressively; 144 (32.8%) patients with early-stage disease and 98 (86.0%) with metastatic disease never had chemotherapy. After a median follow up of 45 months, 5-year overall survival for the whole group was 67.6%. Survival was better for patients with non-metastatic disease (78.8% vs. 25.4%, P<0.001) and for those with node-negative compared to node-positive disease (85.4% vs. 74.1%, P=0.002). On Cox regression, only positive lymph nodes were associated with poor outcome in patients with non-metastatic disease (Hazard Ratio [HR], 1.75; 95% CI: 1.006-3.034, P=0.048). Conclusions : Older Jordanian women with breast cancer present with more aggressive features and advanced-stage disease that reflect poorly on treatment outcomes. Because of comorbidities and poor performance status, some patients were not aggressively treated.

Expression of the Cancer-Testis Antigens, SEMG 1 and Protamine 1, in Early CLL: Their Relationship to Disease Stage and Zap 70 Expression.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4680-4680
Author(s):  
Farouk Meklat ◽  
Yana Zhang ◽  
Zhiqing Wang ◽  
Jian Zhang ◽  
Sukhrob Mustalov ◽  
...  
Keyword(s):  
Vaccine Development ◽  
Tumor Vaccine ◽  
Early Stage ◽  
Disease Stage ◽  
Early Disease ◽  
Early Stage Disease ◽  
Stage Disease ◽  
Ct Antigen ◽  
Cancer Testis ◽  
Protamine 1

Abstract Despite advances in modern chemotherapy, CLL remains incurable. CLL is an indolent disease. It expresses a panel of Cancer-Testis (CT) antigens. CLL leukemia cells are susceptible to the cytotoxicity of T cells. CLL is, therefore, an ideal disease for immunotherapeutic approaches. Immunotherapy, in addition to being less toxic and more specific than chemotherapy, provides a different mode of cytotoxicity that may synergize with that induced by chemotherapeutic agents. Immunotherapy also offers the prospect of inducing immune memory that may be important for long term disease-free survival of patients with CLL. However, there are obstacles that may prevent successful immunotherapy. CLL patients are generally immunosuppressed even before any therapy is given and the immunosuppression increases as the disease progresses. Therefore, any immunotherapeutic approaches for CLL should be aplied in early disease when immunosuppression is least encountered. We previously demonstrated the expression of a CT antigen, SEMG 1, in 3/9 patients with CLL. Furthermore, we also demonstrated that the presence of high titer IgG in the serum of patients expressing SEMG 1, suggesting the in vivo immunogenicity of SEMG 1 in the cancer-bearing autologous host. We have also recently used SEMG 1 as the bait in a yeast two-hybrid system of testicular cDNA library and identified that Protamine 1 is the interacting ligand of SEMG 1 and that Protamine 1 is also a novel CT antigen, suggesting that both SEMG 1 and Protamine 1 may be suitable antigens for tumor vaccine development. However, the expression of SEMG 1 and Protamine 1 in early CLL is unknown. We have in this study set out to determine whether or not SEMG 1 and/or Protamine 1 could be used for the design of tumor vaccine for the targeting of patients with early CLL, in particular, those with poor risk disease, as predicted by Zap 70 expression. Using pairs of sequence-specific primers in RT-PCR on a cohort of CLL (41 Stage 0/I and 6 Stage II/III), we found that SEMG 1 gene is expressed in 24/47 (51%) and Protamine 1 in 16/47 (34%) of CLL patients. Gene expression in most cases was associated with the detection by immunocytochemistry of SEMG 1 and/or Protamine 1 in the CLL cells. The expression frequency of SEMG1 and Protamine 1 in CLL did not appear to differ between early and late stage disease. 19/41 of patients with early stage disease and 5/6 of patients with late disease expressed SEMG 1; 12/41 of patients with early stage disease and 4/6 patients with late disease expressed Protamine 1. Furthermore, the expression of these antigens was equally distributed between Zap 70+ and Zap 70− CLL. SEMG 1 was expressed in 4/6 of Zap 70+ CLL (all 6 had early disease) and 2/9 of Zap 70− CLL (1/8 early disease and 1/1 late disease). Interestingly, although Protamine 1 expression in CLL predicted for SEMG 1 co-expression, only 67% of SEMG 1+ CLL expressed Protamine 1. Our results, therefore, suggest that both SEMG 1 and Protamine 1 are suitable targets for tumor vaccine development for some patients with early CLL, especially those with high risk disease, as predicted by Zap 70 expression.

Neuroendocrine carcinoma of the cervix: A single institution’s experience.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e15585-e15585
Author(s):  
Megan Preston ◽  
Georgia Anne-Lee McCann ◽  
David M. O'Malley ◽  
Christina Boutsicaris ◽  
Larry J. Copeland ◽  
...  
Keyword(s):  
Adjuvant Therapy ◽  
Metastatic Disease ◽  
Survival Data ◽  
Early Stage ◽  
Stage I ◽  
Stage Ii ◽  
Advanced Stage ◽  
Single Institution ◽  
Early Stage Disease ◽  
Stage Disease

e15585 Background: Neuroendocrine carcinomas (NEC) of the cervix comprise only 2% of all cervical cancers. As a result, prospective data is limited and treatment guidelines rely on literature from lung NEC. The objective of this study was to examine and report on our experience in the management of this rare, aggressive disease. Methods: This was an IRB-approved, single-institution, retrospective review. Study criteria included patients with cervical NEC diagnosed between 1990-2011. Demographic, treatment and survival data was collected. Progression-free survival (PFS) and overall survival (OS) was defined as the time from date of initial treatment until progression or death respectively, or date of last contact. Results: A total of 24 patients met inclusion criteria. The median age at diagnosis was 43. Median PFS was 13.6 months and median OS was 16.4 months. The majority of patients had advanced-stage disease (61% stage II-IV, 39% stage I). Of the 9 patients with stage I disease, 4 were treated with platinum + etoposide-based neoadjuvant chemotherapy and 5 were treated with initial radical surgery. Seven of the 9 patients had post-operative adjuvant therapy consisting of chemotherapy, chemo-radiation or radiation only. Seven of the 9 patients (78%) were alive at last follow-up. Of the two patients who were deceased, one had metastatic disease found at surgery and the other declined adjuvant therapy and died of recurrence. Patients with stage II-IV disease (n=15) had a median PFS and OS of 11.5 and 12.1 months, respectively. Only 2 had no evidence of disease at last encounter. The remainder died without achieving remission. Patients with metastatic disease had significantly worse survival when compared to those with loco-regional disease with a median OS of 8 vs. 28 months (p = .03), respectively. Conclusions: We report one of the largest single-institution experiences of neuroendocrine cervical cancer. Advanced-stage patients had a poor prognosis regardless of therapy. However, multi-modality therapy in early-stage disease resulted in an excellent prognosis (78% survival) for these rare, highly aggressive tumors. These findings support the goal of curative intent for early-stage disease using multi-modality therapy.

scholarly journals Recent advances in understanding and managing cutaneous T-cell lymphomas

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 331
Author(s):  
Patrick M. Brunner ◽  
Constanze Jonak ◽  
Robert Knobler
Keyword(s):  
T Cell ◽  
Early Stage ◽  
Disease Stage ◽  
Treatment Modalities ◽  
Early Stage Disease ◽  
T Cell Lymphomas ◽  
Stage Disease ◽  
Related Quality ◽  
Health Related ◽  
Stage Ivb

Cutaneous T-cell lymphomas (CTCLs) comprise a heterogeneous group of extranodal non-Hodgkin lymphomas involving primarily the skin and mycosis fungoides is its most frequent entity. Whereas most patients show an indolent course in early disease (clinical stages IA to IIA), some patients progress to advanced disease (stage IIB or higher), and the 5-year survival rate is unfavorable: only 47% (stage IIB) to 18% (stage IVB). Except for allogeneic stem cell transplantation, there is currently no cure for CTCL and thus treatment approaches are palliative, focusing on patients’ health-related quality of life. Our aims were to review the current understanding of the pathogenesis of CTCL, such as the shift in overall immune skewing with progressive disease and the challenges of making a timely diagnosis in early-stage disease because of the lack of reliable positive markers for routine diagnostics, and to discuss established and potential treatment modalities such as immunotherapy and novel targeted therapeutics.

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