Browsing by Subject "SEZARY-SYNDROME"

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  • Kaartinen, I.; Sunela, K.; Alanko, J.; Hukkinen, K.; Karjalainen-Lindsberg, M. -L.; Svarvar, C. (2017)
    Breast lymphomas comprise a rare group of malignant breast tumors. Among these, a new entity has emerged as a potentially under diagnosed disease. Breast implant-associated anaplastic large cell lymphoma (BI-ALCL) most often manifests as a late periprosthetic effusion between 1 and 10 years after the implantation of silicone or saline-filled breast prostheses. BI-ALCL is an anaplastic lymphoma kinase-negative T-cell lymphoma that has a distinctively different clinical course than other breast lymphomas or ALCLs. Diagnosis is based on aspiration of the effusion around the implant and CD30 positivity of the sample. Every periprosthetic effusion after breast augmentation or reconstruction using implants should be considered as potential BI-ALCL until proven otherwise. The majority of cases at diagnosis are in the in situ stage, i.e., confined to the lumen around the prosthesis. Most patients have an excellent prognosis when complete removal of the capsule and prosthesis with negative margins is achieved surgically. Some patients, however, develop infiltrative disease with a potentially life-threatening clinical course. Treatment planning regarding the extent of surgery and role of adjuvant therapy, especially in advanced cases, requires further investigation. (c) 2017 Elsevier Ltd, BASO-The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.
  • Molloy, K.; Jonak, C.; Woei-A-Jin, F. J. S. H.; Guenova, E.; Busschots, A. M.; Bervoets, A.; Hauben, E.; Knobler, R.; Porkert, S.; Fassnacht, C.; Cowan, R.; Papadavid, E.; Beylot-Barry, M.; Berti, E.; Violetti, S. Alberti; Estrach, T.; Matin, R.; Akilov, O.; Väkevä, L.; Prince, M.; Bates, A.; Bayne, M.; Wachsmuch, R.; Wehkamp, U.; Marschalko, M.; Servitje, O.; Turner, D.; Weatherhead, S.; Wobser, M.; Sanches, J. A.; McKay, P.; Klemke, D.; Peng, C.; Howles, A.; Yoo, J.; Evison, F.; Scarisbrick, J. (2020)
    Background Mycosis fungoides (MF) and Sezary Syndrome (SS) are the most common cutaneous T-cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. Aim To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Methods 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. Results The study population consisted of 237 patients [60 center dot 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 (beta = 8 center dot 61; P = 0 center dot 003) and alopecia (beta = 9 center dot 71, P = 0 center dot 02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2 center dot 14, 95% confidence interval (CI) 1 center dot 19-3 center dot 89] and emotions (OR 1 center dot 88, 95% CI 1 center dot 09-3 center dot 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. Conclusions 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.
  • Maliniemi, Pilvi; Vincendeau, Michelle; Mayer, Jens; Frank, Oliver; Hahtola, Sonja; Karenko, Leena; Carlsson, Emilia; Mallet, Francois; Seifarth, Wolfgang; Leib-Moesch, Christine; Ranki, Annamari (2013)
  • Ranki, Annamari; Vakeva, Liisa; Sipila, Laura; Krohn, Kai (2011)
  • Vakeva, Liisa; Lipsanen, Tuomas; Sintonen, Harri; Ranki, Annamari (2017)
    Cutaneous T-cell lymphomas (CTCL), especially mycosis fungoides, can be considered as a state of longstanding low-grade systemic inflammation. Many studies have focused on secondary cancers with CTCL, but information about comorbidities is limited. A total of 144 patients with CTCL at Helsinki University Central Hospital during 2005 to 2015 were studied to determine associated comorbidities and causes of death in this cohort. Compared with an age-standardized control population, the prevalence of type 2 diabetes mellitus was increased among patients with CTCL with no link to obesity. Patients with CTCL had a lower prevalence of hypertension, myocardial infarction and stroke than the control group. The 3 most common causes of death were CTCL, coronary artery disease and lung cancer. The increased risk of myocardial infarction or stroke reported previously was not detected in this patient group.
  • Väkevä, Liisa; Ranki, Annamari; Hahtola, Sonja (2012)