Epidemiology and status of specialized medical care for patients with cutaneous T-cell lymphomas: A survey of 163 patients of Russian Federation

Background. In the Russian Federation, there are currently no official statistical data concerning the incidence of cutaneous T-cell lymphomas (CTCL) and the prevalence of CTCL due to the absence of isolation rubrics in the headings of lymphoproliferative diseases of the federal state statistical observation of the separate neologies.

Aim. To obtain and analyze the results of the number of patients with CTCL (mycosis fungoides and Sézary syndrome) who were on the dispensary observation in medical organizations of dermatovenereological profile in the period 2015 to 2020, the demographic and clinical-epidemiological characteristics, the applied methods of diagnosis and the therapy.

Materials and methods. To conduct the study, the questionnaire was developed to obtain the information of the number of patients observed with diagnoses of "mycosis fungoides" and "Sézary syndrome" in medical organizations of the dermatovenereological profile of the Federal Subjects of the Russian Federation in the period 2015-2020, were researched the demographic characteristics, the diagnostic methods and the therapy in the studied group of patients.

Results. From 24 Federal Subjects of the Russian Federation were obtained the data concerning 163 patients with CTCL under the observation in medical organizations in the period 2015–2020. From 35 Federal Subjects of the Russian Federation the information showed the absence of patients with CTCL under the observation in medical organizations of dermatovenereological profile. We did not receive the data from 10 Federal Subjects of the Russian Federation. Among 163 patients in 144 (89%) were diagnosed mycosis fungoides, in 17 (10%) – Sézary syndrome and in 2 (1%) patients were diagnosed other variants of CTCL. The data concerning the demographic characteristics, the diagnosis and the therapy of CTCL were obtained in 155 of 167 patients. We showed that in 44% of cases, the disease was detected by dermatovenerologists, and the diagnosis was most often (39%) determined by oncologists. In 49% of cases the diagnosis was determined only on the basis of the results of histological study, the immunohistochemical study and the polymerase chain reaction were used in 33% and in 3% of cases, respectively. From 155 patients, 52% were under dispensary observation by oncologists, 41% by hematologists, 26% of patients were observed by dermatologists; 99 patients were under dispensary observation by only one specialist: 50 (32%) of patients – by oncologist, 40 (26%) of patients – by hematologist, 9 (6%) of patients – by dermatovenerologist. Nine (6%) of patients were under dispensary observation by three specialists. The information concerning the therapy was available in 92 (59%) of 155 patients. The most commonly applied method of treatment was chemotherapy – 72%, phototherapy was received by 26%, the same percentage (18%) was received in two groups: the application of interferon α2b and the application of methotrexate, 1% of patients received radiation therapy. The lethal outcome was registered in 53 (34%) patients. Median duration of the disease from the time of diagnosis to death was 3.5±5.0 years, the median – 2 years, the mode – 1 year.

Conclusion. For the first time, the attempt was made to summarize the data of the number of patients with CTCL, to describe the demographic, clinical and epidemiological characteristics, the data concerning diagnostics and the therapy. The obtained preliminary results are required further detailing investigation in close cooperation with the professional specialists such as hematologists and oncologists.