Cutaneous Lymphoma International Prognostic Index Research Articles

Predictors for the use of systemic therapy in stage IB Mycosis fungoides.

The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is aprospective analysis of an international database. Here we examine front-line treatments and quality of life (QoL) inpatients with newly diagnosed mycosis fungoides (MF). To identify (i) differences in first-line approaches according to tumour-nodes-metastasis-blood (TNMB)staging; (ii) parameters related to a first-line systemic approach and (iii) response rates and QoL measures. In total, 395 newly diagnosed patients with early-stage MF (stage IA-IIA) were recruited from 41 centresin 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review. The most common first-line therapy was skin-directed therapy (SDT) (322 cases, 81·5%), while a smallerpercentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariateanalysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA,20%; IA-IB vs. IIA, P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs.T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first-line SDT was 73%,while the ORR to first-line systemic treatments was lower (57%) (P = 0·027). Health-related QoL improvedsignificantly both in patients with responsive disease and in those with stable disease. Disease characteristics such as presence of plaques and FMF influence physician treatment choices,and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, futuretreatment guidelines for early-stage MF need to address these issues.

Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides.

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is characterized by a variable clinical course, presenting either as indolent disease or showing fatal progression due to extracutaneous involvement. Importantly, the lack of prognostic models and predominantly palliative therapy settings hamper patient care. Here, we aimed to define survival rates, disease prediction accuracy, and treatment impact in MF. Hundred-forty MF patients were assessed retrospectively. Prognosis and disease progression/survival were analyzed using univariate Cox proportional hazards regression model and Kaplan-Meier estimates. Skin tumors were linked to shorter progression-free, overall survival and a 3.48 increased risk for disease progression when compared to erythroderma. The Cutaneous Lymphoma International Prognostic Index identified patients at risk in early-stage disease only. Moreover, expression of Ki-67 >20%, CD30 >10%, CD20+, and CD7- were associated with a significantly worse outcome independent of disease stage. Only single-agent interferon-α and phototherapy combined with interferon-α or retinoids/bexarotene achieved long-term disease control in MF. Our data support predictive validity of prognostic factors and models in MF and identified further potential parameters associated with poor survival. Prospective studies on prognostic indices across disease stages and treatment modalities are needed to predict and improve survival.

P-168 - Blood tumour burden does not correlate with skin tumour burden in early-stage mycosis fungoides (MF): PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) registry analysis

P-168 - Blood tumour burden does not correlate with skin tumour burden in early-stage mycosis fungoides (MF): PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) registry analysis

P54 Blood tumour burden does not correlate with skin tumour burden in early-stage mycosis fungoides: analysis of the PROspective Cutaneous Lymphoma International Prognostic Index international registry

Abstract Blood involvement B1, defined as absolute counts 250–1000 IU of an aberrant CD4+CD7– or CD4+CD26– phenotype, may be found in early-stage mycosis fungoides (MF). There is a suggestion that B1 blood involvement is a poor prognostic marker, but there is limited evidence investigating the prognostic significance of B0 or B1 blood involvement in early-stage MF. Loss of CD26 and/or CD7 expression on CD4 cells may occur in MF; however, it is unclear whether loss of either one of these immunophenotypic markers bears more significance on prognosis. For B2 blood involvement (&amp;gt; 1000 IU), the International Society for Cutaneous Lymphomas (ISCL) criteria define Sézary syndrome as CD26 loss of &amp;gt; 30% or CD7 loss of &amp;gt; 40%. Understanding the impact of B1 and comparative loss of CD26 and/or CD7 in early-stage MF may allow patients at higher risk of stage progression to be identified and selected for better therapeutic options. The aims of this study were to analyse B0 vs. B1 blood involvement at diagnosis in patients with early-stage MF (IA–IIA) and evaluate the relative CD4+CD7– and CD4+CD26– absolute cell counts. Prospective data from the PROCLIPI registry were analysed. Ninety-four patients with early-stage MF were identified from our database, 67 (71%) of whom were B0 [stage IA, n = 23 (34%); stage IB, n = 24 (36%); stage IIA, n = 20 (30%)] and 27 (29%) were B1 [stage IA, n = 12 (44%); stage IB, n = 10 (37%); stage IIA, n = 5 (18%)]. Median age at diagnosis in B0 patients was 59 years (range 20–91) vs. 61 years (range 21–81) in B1 patients. Median CD4+CD7– cell count was 16.4% (range 0–41%) in patients with B0 involvement and 28.1% (range12–45%) in B1 patients. Median CD4+CD26– cell count was 16% (range 0–39.1%) in B0 and 28% (range 0–44%) in B1 patients. The Median Modified Severity-Weighted Assessment Tool score in B1 patients was 11 (range 1–73) vs. 20 (range 0.5–100) in B0 patients. There was no significant difference in progression to the advanced stage between B0 and B1 (P = 0.112) patients or overall survival between B0 and B1 patients during median follow-ups of 49 months and 45 months, respectively. These results suggest that blood tumour burden in early-stage MF, measured by the percentage aberrancy in CD4+CD7– and CD4+CD26– cell counts, does not correlate with clinical stage, skin tumour burden or stage progression. This suggests that in B1 involvement, atypical lymphocytes may lose their skin-homing properties. Interestingly, levels of CD4+7– and CD26– are similar in B1 involvement, despite the ISCL criteria defining B2 involvement as a lower relative loss of CD26–. Identifying B1 patients is important as certain therapeutic options such as extracorporeal photopheresis and mogamulizumab may be indicated in patients with early-stage MF, refractory to skin-directed therapies.

The PROCLIPI international registry, an important tool to evaluate the prognosis of cutaneous T cell lymphomas

PROCLIPI is the PROgnostic Cutaneous Lymphoma International Prognostic Index Study with the main aim to produce a prognostic index in mycosis fungoides (MF) and Sezary syndrome (SS). The study prospectively collects clinical, haematological, pathological, imaging, treatment with responses, quality of life and survival data using careful predefined datasets. Patients are subject to a central review to confirm diagnosis. PROCLIPI opened in 2015 and recruitment has been strong with to date 1916 patients recruited from 52 Centres from 19 countries across 6 continents.

Updated review on prognostic factors in mycosis fungoides and new skin lymphoma trials.

Ten-year survival rates in mycosis fungoides (MF) broadly varies, however, there is no standardized prognostic index available. This is presumably due to low prevalence, heterogeneity, and diagnostic challenges in MF. Recent studies have focused on identifying objective prognostic indices by using different parameters for survival determinants. The Cutaneous Lymphoma International Prognostic Index (CLIPI) and the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) represent prototypical studies that identify prognostic factors, seeking to improve management and outcomes in early-stage MF. Detecting these factors and stratifying MF patients according to their disease progression risk may help to manage these patients more efficiently. Review the current literature to determine the risk factors determining prognosis in MF. A Comprehensive literature search was performed using electronic online databases "PubMed" and "Google Scholar" using key words 'prognostic factor', 'prognostic indicator', 'mycosis fungoides', 'Sezary syndrome', 'Skin Lymphoma', 'Cutaneous Lymphoma'. Articles published in English language were considered for the review. The strongest prognostic factor in MF patients is the stage of the disease. T stage and the presence of extracutaneous disease are the most important factors for survival. Other factors that are associated with worse prognosis are male gender, age >60, presence of plaques, folliculotropism, eosinophilia and lymph node stage above N1/Nx. Elevated LDH was associated with later tumor stages and large cell phenotype at diagnosis had a better prognosis. KIR3DL2 was associated with malignant transformation. The PROCLIPI study has assessed risk factors collected in MF patients from different countries and across different ethnicities following a rigorous clinicopathologic process. The findings presented here illustrated that disease prognosis in early stages depends on many contributing factors. Detection and stratification of such factors may allow a personalized approach to management of these patients.

Qol-O-01 - Experience of a supra-regional cutaneous lymphoma centre during the COVID-19 pandemic

Introduction:Prioritisation of National Health Service (NHS) resources to cope with the COVID-19 pandemic has had a major impact on dermatology services. The consequences of the pandemic on the care of patients with primary cutaneous lymphoma (CL) in the U.K. is unknown.Objective:To examine the impact of the Covid-19 pandemic on patients enrolled in the prospective registration and data collection study for newly diagnosed patients with all stages of MF/SS ‘PROCLIPI (PROspective Cutaneous Lymphoma International Prognostic Index) Study’.Methods:The impact of the COVID-19 pandemic was assessed by comparing the pre-COVID clinical stage (TNMB stage, mSWAT score, global response assessment) and health-related quality of life (HRQoL (Skindex-29) prior to and 4.5 (4.0-5.6) months after the onset of the COVID-19 pandemic. A specific COVID-19 dataset was used to capture the impact of the pandemic on study participants.Results:PROCLIPI study visit data was analysed in 75 patients with a mean (±SD) age of 65.19 (±14.31) years with a male: female ratio of 1.4:1. 61 patients had early stage (IA, n=41; IB, n=17 and IIA, n=3) and 14 had late-stage disease (IIB, n=3; IIIA, n=4; IIIB, n=1; IVA1, n=3 and IVA2, n=3) of whom 70 had MF and five SS. Global response assessment was possible in 40 patients. 17.1% (n=7) had an overall response (CR=1; PR=6), 43.9% (n=18) stable disease and 39% (n=16) progressive disease. The mean (±SEM) time to disease progression was 13.82 (±1.22) months and the median (IQR) mSWAT score increased from 7.25 (0.85–30.25) to 26.4 (2.6–41.78), p=0.08. 12 patients had missed/delayed treatments (phototherapy, n=5; radiotherapy, n=1; topical skin direct therapy, n=3; extracorporeal photopheresis, n=1; investigational medicinal product, n=2). We found no quantitative differences in health-related quality of life (HRQoL) using Skindex-29 (n=75) however 40.6% of patients rated the pandemic as having a moderate to severe impact on their QoL. COVID-19 risk group stratification identified 16.7% of patients with high risk and 54.5% of patients with moderate risk features for severe illness from the virus. Five patients developed COVID-19 illness and one with advanced disease (SS, Stage IVA2) in a high-risk COVID-19 group died.Conclusion:The COVID-19 pandemic has significantly impacted the care of patients with CL. From March 2020 all CL clinics were cancelled, clinical trials were suspended and only emergency appointments permitted. Further work comparing progression status in a matched cohort from 2015 and 2016 will further assess the impact of the COVID-19 pandemic in CL.

Quality of life in patients with mycosis fungoides and Sézary syndrome undergoing low-dose total skin electron beam therapy with or without maintenance therapy

Quality of life in patients with mycosis fungoides and Sézary syndrome undergoing low-dose total skin electron beam therapy with or without maintenance therapy

Treatment of early-stage mycosis fungoides: results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study.

The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is a prospective analysis of an international database. Here we examine front-line treatments and quality of life (QoL) in patients with newly diagnosed mycosis fungoides (MF). To identify (i) differences in first-line approaches according to tumour-nodes-metastasis-blood (TNMB) staging; (ii) parameters related to a first-line systemic approach and (iii) response rates and QoL measures. In total, 395 newly diagnosed patients with early-stage MF (stage IA-IIA) were recruited from 41 centres in 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review. The most common first-line therapy was skin-directed therapy (SDT) (322 cases, 81·5%), while a smaller percentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariate analysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA, 20%; IA-IB vs. IIA,P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs. T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first-line SDT was 73%, while the ORR to first-line systemic treatments was lower (57%) (P = 0·027). Health-related QoL improved significantly both in patients with responsive disease and in those with stable disease. Disease characteristics such as presence of plaques and FMF influence physician treatment choices, and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, future treatment guidelines for early-stage MF need to address these issues.

Should we be imaging lymph nodes at initial diagnosis of early-stage mycosis fungoides? Results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) international study.

Early-stage mycosis fungoides (MF) includes involvement of dermatopathic lymph nodes (LNs) or early lymphomatous LNs. There is a lack of unanimity among current guidelines regarding the indications for initial staging imaging in early-stage presentation of MF in the absence of enlarged palpable LNs. To investigate how often imaging is performed in patients with early-stage presentation of MF, to assess the yield of LN imaging, and to determine what disease characteristics promoted imaging. A review of clinicopathologically confirmed newly diagnosed patients with cutaneous patch/plaque (T1/T2) MF from PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) data. PROCLIPI enrolled 375 patients with stage T1/T2 MF: 304 with classical MF and 71 with folliculotropic MF. Imaging was performed in 169 patients (45%): 83 with computed tomography, 18 with positron emission tomography-computed tomography and 68 with ultrasound. Only nine of these (5%) had palpable enlarged (≥ 15 mm) LNs, with an over-representation of plaques, irrespectively of the 10% body surface area cutoff that distinguishes T1 from T2. Folliculotropic MF was not more frequently imaged than classical MF. Radiologically enlarged LNs (≥ 15 mm) were detected in 30 patients (18%); only seven had clinical lymphadenopathy. On multivariate analysis, plaque presentation was the sole parameter significantly associated with radiologically enlarged LNs. Imaging of only clinically enlarged LNs upstaged 4% of patients (seven of 169) to at least IIA, whereas nonselective imaging upstaged another 14% (24 of 169). LN biopsy, performed in eight of 30 patients, identified N3 (extensive lymphomatous involvement) in two and N1 (dermatopathic changes) in six. Physical examination was a poor determinant of LN enlargement or involvement. Presence of plaques was associated with a significant increase in identification of enlarged or involved LNs in patients with early-stage presentation of MF, which may be important when deciding who to image. Imaging increases the detection rate of stage IIA MF, and identifies rare cases of extensive lymphomatous nodes, upstaging them to advanced-stage IVA2.

A study of quality of life in people with mycosis fungoides and Sézary syndrome

Mycosis fungoides (MF) is the commonest type of skin lymphoma and is part of a group of conditions known as cutaneous T-cell lymphomas (CTCL). MF can appear as flat/raised, smooth/scaly red lesions known as patches and plaques, making it difficult to tell apart from eczema or psoriasis. Raised “lumps” called tumours can appear and the disease can become more serious. Sézary syndrome (SS) is a leukaemic form of CTCL and has an extensive red, itchy rash over most of the body, the presence of cancerous cells in the blood, and enlarged lymph glands. MF/SS can reduce patient quality of life (QoL) due to symptoms including itch and pain, poor self-esteem secondary to cosmetic disfigurement, and can impair interpersonal relationships. In this article, we examine QoL in patients with MF/SS in a large international research study called PROCLIPI, using a questionnaire called Skindex-29 which asks patients to comment on skin related questions which may affect their life enjoyment. We identified a number of important determinants of worse QoL in MF/SS including: female gender, patients diagnosed with SS and those with more widespread disease. Disease features which were associated with more impaired QoL included alopecia (hair loss) and confluent erythema (red patches and plaques merging together to form larger areas of affected skin). Combining all these factors together, we found that female patients, alopecia and confluent erythema were the most important. Given that females had worse QoL, we examined more closely which items in the Skindex-29 questionnaire differed between males and females. Female patients experienced more burning, stinging, itch and irritation on their skin and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. Our research study has highlighted important aspects of QoL in MF/SS and emphasizes the urgent need for a more comprehensive QoL questionnaire to address not only the skin related aspects of the disease, but also the effects of cancer and its treatment on patient QoL. This is a summary of the study: Characteristics associated with significantly worse quality of life in mycosis fungoides/Sézary syndrome from the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study

Survival in Mycosis Fungoides and Sezary Syndrome: How Can We Predict Outcome?

Early-stage mycosis fungoides (MF) has been associated with long survival. A recent meta-analysis including 6,279 patients with MF and Sezary syndrome found that about 10-20% of stage IB patients don't survive 5 years, whereas patients with advanced-stage MF and Sezary syndrome have a 5-year survival chance of about 20-60%. Identifying prognostic markers to better identify those at risk of limited survival may allow improved management choices and this, coupled with newer treatments, could improve survival.

Characteristics associated with significantly worse quality of life in mycosis fungoides/Sézary syndrome from the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study.

Mycosis fungoides (MF) and Sézary Syndrome (SS) are the most common cutaneous T-cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex-29 questionnaire. Skindex-29 scores were analysed in relation to patient- and disease-specific characteristics. The study population consisted of 237 patients [60·3% male; median age 60 years, (interquartile range 49-70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex-29, was worse in women, SS, late-stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (β = 8·61; P = 0·003) and alopecia (β = 9·71, P = 0·02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2·14, 95% confidence interval (CI) 1·19-3·89] and emotions (OR 1·88, 95% CI 1·09-3·27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex-29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated cutaneous T-cell lymphoma-specific questionnaire is urgently needed to more accurately assess disease-specific HRQoL in these patients. What's already known about this topic? Cross-sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sézary syndrome (SS) have shown significant impairment in health-related quality of life (HRQoL). Previous studies on assessing gender-specific differences in HRQoL in MF/SS are conflicting. More advanced-stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS. In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema. MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of cutaneous symptoms, as well as a negative impact on emotional well-being.

176 The Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) Study identifies clinical prognostic markers and establishes the foundation for large-scale translational research in cutaneous lymphoma

176 The Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) Study identifies clinical prognostic markers and establishes the foundation for large-scale translational research in cutaneous lymphoma

The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients.

Survival in mycosis fungoides (MF) is varied and may be poor. The PROCLIPI (PROspective Cutaneous Lymphoma International Prognostic Index) study is a web-based data collection system for early-stage MF with legal data-sharing agreements permitting international collaboration in a rare cancer with complex pathology. Clinicopathological data must be 100% complete and in-built intelligence in the database system ensures accurate staging. To develop a prognostic index for MF. Predefined datasets for clinical, haematological, radiological, immunohistochemical, genotypic, treatment and quality of life are collected at first diagnosis of MF and annually to test against survival. Biobanked tissue samples are recorded within a Federated Biobank for translational studies. In total, 430 patients were enrolled from 29 centres in 15 countries spanning five continents. Altogether, 348 were confirmed as having early-stage MF at central review. The majority had classical MF (81·6%) with a CD4 phenotype (88·2%). Folliculotropic MF was diagnosed in 17·8%. Most presented with stage I (IA: 49·4%; IB: 42·8%), but 7·8% presented with enlarged lymph nodes (stage IIA). A diagnostic delay between first symptom development and initial diagnosis was frequent [85·6%; median delay 36 months (interquartile range 12-90)]. This highlights the difficulties in accurate diagnosis, which includes lack of a singular diagnostic test for MF. This confirmed early-stage MF cohort is being followed-up to identify prognostic factors, which may allow better management and improve survival by identifying patients at risk of disease progression. This study design is a useful model for collaboration in other rare diseases, especially where pathological diagnosis can be complex.

Quality of life in patients with mycosis fungoides and Sezary syndrome is significantly worse in female patients, Sézary syndrome and those with more extensive skin involvement

Background: Patients with Mycosis fungoides/Sézary Syndrome (MF/SS) have a poor quality of life (QoL) and a combination of skin, pruritus and cancer QOL questionnaires are usually used in combination in clinical trials. The Skindex-29 is a self-administered questionnaire to comprehensively measure the effects of skin disease on patient's quality of life (QoL). We sought to evaluate QOL in patients with MF/SS enrolled in the PROCLIPI (Prospective Cutaneous Lymphoma International Prognostic Index) study using Skindex-29.

The PROCLIPI study; a prototype registry for rare disease with global collaboration for establishment of a prognostic index in mycosis fungoides and Sezary syndrome

The PROCLIPI Study, a multi-national PROspective Cutaneous Lymphoma International Prognostic Index study to develop a prognostic index in mycosis fungoides (MF) and Sezary syndrome (SS) was launched in 2015. This study is collecting pre-defined clinical, haematological, radiological, immunohistochemical, genotypic, quality of life and treatment data using unique database systems designed for PROCLIPI. Central clinical and pathological review is performed to confirm diagnosis and stage.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

BackgroundMycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed.ObjectiveTo evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF.MethodsThis is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death.ResultsOne hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification.Study LimitationsSmall sample and its retrospective analysis.ConclusionsSince CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

Age as a potential new prognostic indicator in primary cutaneous B-cell lymphoma (PCBCL).

e19034 Background: PCBCL is a group of rare lymphoproliferative disorders, with an estimated annual incidence of 2.5 per 1,000,000 persons. Indolent subtypes include Primary Cutaneous Marginal Zone Lymphoma (PCMZL) and Primary Cutaneous Follicular Center Lymphoma (PCFCL). Primary Cutaneous Diffuse Large B-cell Lymphoma (PCDLBCL) is an aggressive subtype with a fatality rate of 50%. The Cutaneous Lymphoma International Prognostic Index (CLIPI) can risk-stratify indolent subtypes, but age is not considered. Here we present our single-institutional analysis of clinicopathologic features and outcomes of patients with PCBCL. Methods: This is a retrospective study of patients evaluated at Moffitt Cancer Center between 01/1990 and 12/2016. Patients were identified using our PCBCL database and diagnosis was verified by independent hematopathologists and dermatopathologists. Staging was determined by ISCL/EORTC criteria. Demographics, subtype, stage, disease course, and CLIPI scores were collected. Continuous and categorical values were tested using Kruskal-Wallis ANOVA method and Fisher’s Exact Test, respectively. Kaplan-Meier curves were produced to determine PFS. Results: We identified 37 patients who met diagnostic criteria for PCBCL (35% PCFCL, 40.5% PCMZL, 13.5% PCDLBCL, and 11% indolent, unspecified). Male:female ratio was 2.4:1. 51% of patients were ≥ 60 years old (yo), and 49% were &lt; 60 yo. 54% had stage T1 disease, 27% T2, and 19% T3. Median PFS for patients &lt;60 was 1.1 years, but was not reached for those ≥60. Mean follow-up time was 2.6 years for all patients. Log-rank test showed a statistically significant difference in PFS between the two age groups (p=0.01). PFS for stage of indolent subtypes showed marginal significance (p &lt;0.06). CLIPI for indolent subtypes did not show a significant difference in PFS. Conclusions: We found that age is a highly statistically significant prognostic parameter in PCBCL, as patients ≥ 60 yo had a longer PFS compared to younger patients, even after adjusting for stage and CLIPI. These results are promising for age as a possible prognostic indicator for PCBCL, but validation is needed with a larger sample size.

Prognostic indicators for mycosis fungoides in a Greek population.

Mycosis fungoides (MF) is an indolent cutaneous lymphoma with excellent prognosis at early stages and much poorer outcome during disease progression. Old age, male sex and folliculotropism have been proposed as relevant prognostic factors; however, their exact effect remains debatable. To evaluate MF prognostic indicators and survival rates in a Greek population. Prognostic variables affecting survival rates were studied in 473 patients with MF diagnosed and treated by two academic referral centres in Greece. Kaplan-Meier estimates were used to determine survival rates and progression. The Cox proportional hazards regression model was used to assess prognostic factors. The mean age of diagnosis was 61·7years (SD 16·33). Five-year disease-specific survival was 96% in patients with stage IA disease and 52% in patients with stage IIB disease. Univariate analysis certified that large-cell transformation, clonal rearrangements of the TCR gene, severe pruritus and presence of plaques were the most important prognostic factors. Folliculotropism altered disease progression only in patients with early-stage disease. The application of the Cutaneous Lymphoma International Prognostic Index (CLIPI) on our late-stage group failed to provide reliable evidence. The current Cutaneous Lymphoma International Consortium (CLIC) prognostic index can efficiently distinguish a low-risk from a high-risk group of patients. Tumour-Node-Metastasis-Blood (TNMB) staging was the most important prognostic factor for survival rates in multivariate analysis. In our study we validated the current prognostic indicators for MF in a Greek population and identified new potential prognostic factors for survival outcome. Our findings contribute to the ongoing investigation of prognostic indicators of MF, further validation of which is highly needed through prospective studies and among different populations.