The Prognostic Value of ТР53 Gene Mutations in Patients with Cutaneous T-Cell Lymphomas

BACKGROUND. Primary cutaneous lymphomas represent a heterogeneous group of neoplastic diseases with unique clinical, immunomorphological, and molecular genetic features. The prognosis of cutaneous T-cell lymphomas (CTCL) depends primarily on the tumor variant. For instance, at the early stages of mycosis fungoides the median overall survival is 12 years, whereas in Sezary syndrome it does not exceed 12 months. Within the well-known range of prognostic factors, the role of TP53 gene abnormalities in cutaneous lymphomas remains underresearched.

AIM. To assess the incidence and prognostic value of TP53 gene mutations in different CTCL variants.

MATERIALS & METHODS. The present study enrolled 20 patients with confirmed mycosis fungoides (n = 16) and Sezary syndrome (n = 4). The study was carried out at the molecular biology laboratory of the National Research Center for Hematology. TP53 gene mutations were analyzed by next-generation sequencing with amplification of exons 4–10. Bioinformatic analysis was performed using the standard tools. The 17p13 deletion was detected by FISH.

RESULTS. TP53 gene mutations were identified in 2 (10 %) out of 20 patients, both reported in Sezary syndrome (2/4). In mycosis fungoides patients, no TP53 mutations were detected. Sezary syndrome patients, whether with or without TP53 mutations, showed an aggressive course of the disease (death of 3 patients was registered, the fourth one had been still followed-up by the time of the article submission).

CONCLUSION. Under the present study, TP53 gene mutations were identified only in Sezary syndrome patients, which suggests their association with the most aggressive CTCL variants. However, a small number of studied cases is not sufficient to claim that TP53 gene mutations can be regarded as an independent poor prognostic factor for primary cutaneous lymphomas.

• Liliya Gamilevna Gorenkova National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 https://orcid.org/0000-0002-3967-9183 (unauthenticated)
• B.V. Biderman National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 https://orcid.org/0000-0002-6253-3334 (unauthenticated)
• A.K. Smolianinova National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 https://orcid.org/0000-0003-0591-2589 (unauthenticated)
• E.E. Zvonkov National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 https://orcid.org/0000-0002-2639-7419 (unauthenticated)

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