Retrospective Analysis of 118 Patients With Cutaneous T-Cell Lymphomas: A Single-Center Experience

Abstract

Cutaneous T-cell lymphomas (CTCL) represent rare non-Hodgkin lymphomas (NHL) with an incidence less than 1 per 100,000 inhabitants. The most common type of CTCL is mycosis fungoides (MF), which represents approximately 60% of all CTCL, followed by Sézary syndrome (SS), approximately 5%. We retrospectively analyzed the outcome of 118 patients with MF (n=96) and SS (n=22) treated between the years 1998 and 2021 at the Charles University General Hospital in Prague, Czech Republic. The ratio between men and women was 1.2:1 (62 men, and 56 women). The median age at diagnosis was 62 years (23 to 92 years). From the MF cohort 48 patients (50% out of MF cohort) presented with advanced stage disease. Ninety patients (77%) received a systemic treatment at any time from the diagnosis; the median number of therapy lines was two. At the time of database lock, the overall survival (OS) of 96 patients with MF reached 17.7 years with the median follow-up 4.0 years. With the median follow-up 2.6 years, the median OS of 22 patients with SS was 3.5 years. The most common type of systemic therapy for MF included low-dose methotrexate (61%), interferon-alpha (58%), bexarotene (28%), and chlorambucil (25%). The most common type of therapy for SS included bexarotene (64%), extracorporeal photopheresis (50%), and interferon-alpha (45%). Only the minority of patients received innovative targeted agents including brentuximab vedotin, mogamulizumab, or pembrolizumab. Besides the retrospective analysis of the CTCL cohort, current standards and future perspectives of selected innovative agents are summarized and discussed. The analyzed cohort represents the largest cohort of CTCL patients in the Czech Republic. Overall, the survival parameters of our CTCL cohort are comparable to those previously published by other groups. In conclusion, our analysis of 118 real world cohort of consecutive CTCL patients treated at the single center confirmed the efficacy of immune response modifiers and underlines the urgent need for ample implementation of innovative agents and their combinations into earlier lines of therapy.

Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides

“…In MF, treatment responses are usually short-lived resulting in multiple treatment lines, associated with shorter survival. 48 We are aware of the limitations of this study with its retrospective design and the small sample size, especially for advanced-stage patients. However, it is a representative collective of patients treated at a European expert center in which Ki-67 >20%, CD30 + >10%, CD20 + , and CD7were clearly identified as poor prognostic factors in MF independent of disease stage.…”

“…It can reflect the duration of the therapeutic effect, as well as patients' experiences with treatment tolerability and compliance. 46 It has also been retrospectively assessed in MF, [47][48][49] showing chemotherapy associated with the shortest mean TTNT of 5.1 months 49 and with a higher risk of death if given first-line. 50 The latter finding is in line with our observation, as first-line treatment with chemotherapy was associated with a significant inferior OS and associated with the worst TTNT of 6 months with 82.5% of patients receiving further treatment after 2 years.…”

“…Notably, median TTNT of all therapies within our cohort was 17 months for a first‐line treatment and only 4 months for third‐line, respectively. In MF, treatment responses are usually short‐lived resulting in multiple treatment lines, associated with shorter survival 48 …”

Mortalität, prognostische Parameter und Behandlungsstrategien bei Mycosis fungoides

“…Die TTNT kann die Dauer der therapeutischen Wirksamkeit reflektieren und darüber hinaus die individuellen Erfahrungen der Patienten mit der Verträglichkeit der Behandlung und der Compliance widerspiegeln 46. Einzelne retrospektive Studien[47][48][49] haben die TTNT bei MF untersucht und gezeigt, dass eine Chemotherapie sowohl mit der kürzesten mittleren TTNT von 5,1 Monaten 49 sowie mit einer höheren Mortalität bei Erstlinienbehandlung assoziiert ist 50. Dies deckt sich mit unserer Beobachtung, dass die Erstlinientherapie mit Chemotherapie mit einem deutlich schlechteren OS und der kürzesten TTNT von 6 Monaten verbunden war und 82,5% der Patienten nach 2 Jahren eine weitere Behandlung benötigten.…”

“…Bemerkenswert ist, dass die mediane TTNT aller Therapien in unserer Kohorte 17 Monate für eine Erstlinienbeziehungsweise nur 4 Monate für eine Drittlinientherapie betrug. In der Regel ist das Ansprechen auf eine Behandlung bei MF-Patienten von kurzer Dauer, was oftmals zu mehreren Behandlungslinien führt und mit einer kürzeren Überlebenszeit assoziiert ist 48. Diese Studie weist aufgrund ihres retrospektiven Designs und der geringen Fallzahl, insbesondere bei Patienten im fortgeschrittenen Stadium, einige Limitationen auf.…”