ABSTRACT
IFN a with its cytotoxic and immunological effects on tumorous T cells has been introduced in the treatment of cutaneous T-cell lymphoma since the 1980s. The reported experience (average response rates 45–80%, further improved with combination schemes) is mostly based on the use of the recombinant IFN a-2a or IFN a-2b. However, these interferon formulations are no longer commercially available in Europe and therefore, the pegylated analogue (pegylated IFN a-2a, Pegasys;Hoffmann-La Roche) has been currently used to treat CTCLs. The purpose of the current study was to evaluate the efficacy and safety profile of pegylated IFN a-2a in the treatment of mycosis fungoides (MF). We collected data from three referral Cutaneous Lymphoma Units in Greece, covering a 2-year period, before COVID -19 pandemic restrictions. The primary endpoint was to determine the effectiveness of peg IFN as measured by the overall response rate (RR) in this cohort. Secondary endpoints were difference in RRs with respect to gender, disease stage, presence of folliculotropism and treatment features, time to best response, duration of response, drug survival, reasons for drug discontinuation and safety profile. Statistical analysis of the data was performed using the Statistical Package for Social Sciences (SPSS), version 15.0 (SPSS, Inc., Chicago, IL, USA). Overall, 31 patients were included. Most patients (n=12;38.7%) had ΙΒ-stage disease at peg IFN initiation. In all, 11 (35.5%) patients received PegIFN monotherapy, while 20 (64.5%) subjects on peg IFN concomitantly received bexarotene, acitretin, methotrexate or topical chemotherapy. PegIFN was administered in the majority of patients as third or fourth line therapy (21/31). A 54.8% (17/31) overall response rate was noted: 9.7% and 45.2% for CR and PR, respectively. In our cohort, no differences in RRs with respect to gender (p=0.427), disease stage (p=0.179) or presence of folliculotropism (p=0.532) was observed. Peg IFN was not more effective as monotherapy than as a subsequent agent with respect to overall response (p=0.680). Mean time to best response was 7.29±4.99 months. Treatment dose was reduced in 8 (25.8%) cases due to drug intolerance. Adverse effects were recorded in 21 (67.7%) cases with leukopenia (n=16;76.1%), fatigue (n=9;42.8%) and anemia (n=4;19.0%) being the most recorded. Overall, peg IFN may be considered an effective additional regimen in the treatment of MF, stage IB or higher, with a rather good tolerance and safety profile. [ABSTRACT FROM AUTHOR] Copyright of European Journal of Cancer is the property of Pergamon Press - An Imprint of Elsevier Science and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
ABSTRACT
Background: Erdheim-Chester disease (ECD) is a rare hematopoietic neoplasm of histiocytic origin characterized by an insidious course. The coronavirus disease 2019 (COVID-19) pandemic has put an enormous strain on healthcare systems worldwide both directly and indirectly, resulting in the disruption of healthcare services to prevent, diagnose and manage non-COVID-19 disease. Case Presentation: We describe the case of a 58-year-old male patient with sporadic episodes of self-resolving mild fever and anemia of chronic disease with onset two years before the current presentation. Positron emission/computed tomography scan revealed the presence of moderately hypermetabolic perirenal tissue masses. In order to achieve diagnosis, repeated perirenal tissue biopsies were performed, and the diagnostic evaluation was complicated by the strain put on the healthcare system by the COVID-19 pandemic. The patient contracted SARS-CoV-2 and required hospitalization, but recovered fully. No further ECD target organ involvement was documented. Treatment options were presented, but the patient chose to defer treatment for ECD. Conclusion: A high index of suspicion and multidisciplinary team collaboration is paramount to achieve diagnosis in rare conditions such as ECD. Disruptions in healthcare services in the pandemic milieu may disproportionately affect people with rare diseases and further study and effort is required to better meet their needs in the pandemic setting.