Epidemiological and clinical parameters of cutaneous T-cell lymphoma (based on the register of the Russian Society of Dermatovenerologists and Cosmetologists)

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OBJECTIVE

To study the prevalence of cutaneous skin T-cell lymphoma (mycosis fungoides and Sézary syndrome) according to the registry of the Russian Society of Dermatovenereologists and Cosmetologists, as well as to describe demographic and clinical characteristics, diagnosis and treatment of these patients.

MATERIAL AND METHODS

Analysis of epidemiological and clinical data was based on the register of patients with cutaneous T-cell lymphomas. We assessed demographic indicators (mean age of patients, male-to-female ratio), characteristics of disease, data on diagnosis and treatment. Prevalence of cutaneous T-cell lymphoma in constituent entity of the Russian Federation was calculated as the number of patients per 100.000 population in certain constituent entity.

RESULTS

Prevalence of cutaneous T-cell lymphomas in constituent entities of the Russian Federation ranges from 0.02 to 1.73 per 100.000. The registry contains information about 130 patients: 124 (95.4%) with mycosis fungoides (C84.0) and 6 (4.6%) with Cesari syndrome (C84.1) from 29 (34.1%) constituent entities of the Russian Federation. The diagnosis with stage of disease was indicated in 100 (76.9%) patients, and stage II A was the most common (42 (42.0%) patients). Data on specialists who first established the diagnosis were available in 106 (81.5%) patients. Dermatovenereologists diagnosed 46 (43.4%) cases, oncologists — 37 (34.9%) patients, hematologists — 23 (21.7%) patients. Cutaneous T-cell lymphoma was confirmed by laboratory methods in 117 (90.0%) patients. Of these, histological examination was performed in 107 (91.4%) patients, immunohistochemical analysis — in 46 (39.3%) patients, molecular genetic research of T-cell clonality — in 23 (19.6%) patients. Information on therapy was available for 103 (79.2%) people. 55 out of 103 (53.4%) patients underwent systemic drug therapy, 47 (45.6%) — chemotherapy, 46 (44.7%) — phototherapy. According to the latest analysis of database, 33 (25.4%) patients with cutaneous T-cell lymphoma died (19 (57.6%) due to underlying disease, 14 (42.4%) due to other causes).

Keywords:

cutaneous T-cell lymphoma

mycosis fungoides

Sézary syndrome

epidemiology

demographic characteristics

Authors:

Kubanov A.A.

State Research Center of Dermatovenerology and Cosmetology of the Ministry of Healthcare of the Russian Federation

Scopus AuthorID: 6506502738
ResearcherID: L-3008-2014
ORCID: 0000-0002-7625-0503

Rakhmatulina M.R.

State Research Center for Dermatovenereology and Cosmetology

ORCID: 0000-0003-3039-7769

Karamova A.E.

State Research Center for Dermatovenereology and Cosmetology

ORCID: 0000-0003-3805-8489

Vorontsova A.A.

State Research Center for Dermatovenereology and Cosmetology

ORCID: 0000-0002-3129-0050

Novoselova E.Yu.

State Research Center for Dermatovenereology and Cosmetology

ORCID: 0000-0003-1907-2592

Received:

24.08.2023

Accepted:

20.09.2023

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