Sezary syndrome

Current Opportunities for Treatment Optimization of Mycosis Fungoides and Sezary Syndrome

NOVEL DRUGS , , ,

NG Chernova1, OA Kolomeitsev2

1 National Medical Hematology Research Center, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

2 NN Blokhin National Medical Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

For correspondence: Natal’ya Gennad’evna Chernova, PhD, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; Tel.: +7(495)612-48-10; е-mail: ngchernova@mail.ru

For citation: Chernova NG, Kolomeitsev OA. Current Opportunities for Treatment Optimization of Mycosis Fungoides and Sezary Syndrome. Clinical oncohematology. 2018;11(1):34–41.

DOI: 10.21320/2500-2139-2018-11-1-34-41

ABSTRACT

Aim. To estimate vorinostat efficacy in patients with relapsed/refractory mycosis fungoides and Sezary syndrome.

Materials & Methods. The total of 21 patients with refractory and progressive mycosis fungoides and Sezary syndrome receiving vorinostat 400 mg once daily were followed up from 2014 to 2017. The median age was 62 years (range 34–79). The male to female ratio was 10/11. The median number of various regimens of pre-study systemic treatment was 4 (range 1–7). Progressive disease were observed in 85,7 % of patients before administration of vorinostat and after mono- and polychemotherapy.

Results. The study group included 15 patients with mycosis fungoides and 6 patients with Sezary syndrome. Early stages of primary cutaneous T-cell lymphoma were diagnosed in 4 patients, the advanced stages in 17 patients. Seventeen patients received vorinostat treatment in monoregime; 4 patients were administered with vorinostat in combination with methotrexate or α-interferon. The median duration of vorinostat therapy was 6 months (range 1–38). Stabilization of the disease was observed in 47.6 % of cases, response to treatment in 38.1 % of cases (with 5 cases of complete response and 3 cases of partial response), and 14.3 % of patients had progression of the disease. The decrease of skin itching was reported in 38.1 % of patients; skin itching completely resolved in 28.6 % of cases. The adverse events required the vorinostat dose adjustment in 3 cases and treatment discontinuation in 3 cases. The total of 9 patients continue to receive vorinostat.

Conclusion. Vorinostat treatment was shown to be effective in patients with refractory and advanced mycosis fungoides and Sezary syndrome not responding to various types of external, mono- and polychemotherapy. The therapy with vorinostat was associated with higher life expectancy and improved quality of life.

Keywords: vorinostat, mycosis fungoides, Sezary syndrome, refractory and advanced stages, primary cutaneous T-cell lymphoma.

Received: August 18, 2017

Accepted: December 1, 2017

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REFERENCES

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Treatment of Drug-Resistant Mycosis Fungoides and Sezary Syndrome

LYMPHOID TUMORS , , , ,

LG Gorenkova, EA Penskaya, SK Kravchenko, AM Kovrigina, TN Moiseeva, AI Vorob’ev

Hematology Research Center, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Liliya Gamilevna Gorenkova, PhD, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; 8(495)612-23-61; e-mail: l.aitova@mail.ru

For citation: Gorenkova LG, Penskaya EA, Kravchenko SK, et al. Treatment of Drug-Resistant Mycosis Fungoides and Sezary Syndrome. Clinical oncohematology. 2017;10(3):366–71 (In Russ).

DOI: 10.21320/2500-2139-2017-10-3-366-371

ABSTRACT

Background. The most common diseases among cutaneous T-cell lymphomas are mycosis fungoides (MF) and its leukemic variant Sezary Syndrome (SS). These malignant tumors have a progressive character course. To date, no therapy for these diseases has proven effective, especially in the late stages of the disease.

Aim. We aimed to assess the effectiveness of treating resistant forms of MF/SS with prolonged gemcitabine regimen after initial treatment failure.

Materials & Methods. The study included 14 patients with drug-resistant forms of MF/SS (10 patients with MF, 3 patients with SS, and 1 patient with the transformation of MF into large cell lymphoma). The median age was 62 years (range 34–78 years). The study population included 9 males and 7 females. Gemcitabine was administered at a dosage of 250 mg/m2 as a 6–8 hour IV infusion weekly, in a cycle of 21–28 days.

Results. The overall response was 79 % (29 % of patients with complete remission, 50 % of patients with partial remission). In 21 % of patients, the treatment results met the criteria for tumor stabilization. The disease progression was observed in 2 (14 %) patients. This study demonstrates the potential of using prolonged gemcitabine regimen in patients of different age groups with advanced resistant MF/SS who received at least two courses of previous ineffective therapy. In order to evaluate long-term results, further research is needed.

Conclusion. The administration of prolonged gemcitabine regimen may be a treatment of choice in resistant forms of MF/SS after initial treatment failure in different age groups including elderly patients.

Keywords: mycosis fungoides, Sezary syndrome, treatment of drug-resistant forms, gemcitabine, prolonged infusion.

Received: December 6, 2016

Accepted: March 17, 2017

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