Systemic Treatment Options for Advanced-Stage Mycosis Fungoides and Sézary Syndrome

Photiou, Louise; Weyden, Carrie van der; McCormack, Christopher; Prince, H. Miles

doi:10.1007/s11912-018-0678-x

Cited by 35 publications

(37 citation statements)

References 145 publications

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“…In view of the rarity of CTCL, randomized clinical studies are lacking, and treatment is based mainly on the recently published EORTC guidelines 5. This lack of evidence-based data leads to heterogeneity of treatment approaches, especially between US and non-US centers and between institutes 8. It is important to note that there is currently no curative treatment for MF (except for allogeneic stem-cell transplantation).…”

Section: Management Of Mycosis Fungoidesmentioning

confidence: 99%

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Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL): focus on chlormethine gel

Denis¹,

Bénéton²,

Laribi³

et al. 2019

CMAR

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Mycosis fungoides (MF) is a low-grade cutaneous lymphoma accounting for more than half of primary cutaneous T-cell lymphomas (CTCLs). Due to the rarity of CTCL, randomized studies are lacking, and treatment is based mainly on the recent published European Organisation for Research and Treatment of Cancer guidelines. Basically, early-stage MF is treated with skin-directed treatments, whereas advanced-stage MF requires more aggressive therapies. Among the skin-directed therapies, nitrogen mustard has been used for more than 50 years. A gel formulation was developed recently, showing a slight decrease in efficacy, counterbalanced by better tolerance (essentially due to a decrease in delayed hypersensitivity reactions). This review aims to summarize the current management of MF and the role of chlormethine gel in the treatment of the disease.

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Section: Management Of Mycosis Fungoidesmentioning

confidence: 99%

“…It mostly presents with erythema, together with lymphadenopathy and blood involvement with Sézary cells. The prognosis is poor, with median survival of <3 years 8…”

Section: Introductionmentioning

confidence: 99%

Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL): focus on chlormethine gel

Denis¹,

Bénéton²,

Laribi³

et al. 2019

CMAR

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“…ECP has been established as a first-line treatment in CTCL patients with blood involvement (stage IVA1 or IVA2) and erythrodermic stage IIIA or IIIB (30, 32, 33). Treatment recommendations stated 2-weekly cycles of treatment on 2 consecutive days for at least 3 months and subsequent treatment every 3–4 weeks.…”

Section: Indicationsmentioning

confidence: 99%

Extracorporeal Photopheresis—An Overview

2018

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Extracorporeal photopheresis (ECP) has been in clinical use for over three decades after receiving FDA approval for the palliative treatment of the Sézary Syndrome variant of cutaneous T-cell lymphoma (CTCL) in 1988. After the first positive experiences with CTCL, additional indications have been successfully explored including areas such as graft-vs.-host disease (GVHD), scleroderma, and solid organ transplantation. The mechanism of action is still not fully resolved, but important steps in understanding ECP in recent years have been very informative. Originally, the primary hypothesis stated that psoralen and ultraviolet A (UVA) in combination induce apoptosis in the treated immune cells. This view shifted in favor of dendritic cell initiation, modification of the cytokine profile and stimulation of several T-cell lineages, in particular regulatory T-cells. A number of ECP guidelines have been produced to optimize treatment regimens in the clinical context. In CTCL, enough evidence is available for the use of ECP as a first line treatment for Sézary Syndrome (SS), but also as a second line or rescue treatment in therapy-refractory forms of mycosis fungoides (MF). ECP in the treatment of acute and chronic GVHD has shown promising results as second line therapy in steroid-refractory presentations. In solid organ transplantation, ECP has been used to increase tissue tolerance and decrease infections with opportunistic pathogens, attributed to the use of high doses of immunosuppressive medication. Infection with cytomegalovirus (CMV) remains a limiting factor affecting survival in solid organ transplantation and the role of ECP will be discussed in this review. A trend toward prophylactic use of ECP can be observed and may further contribute to improve the outcome in many patients. To further deepen our knowledge of ECP and thus facilitate its use in patients that potentially benefit most from it, future prospective randomized trials are urgently needed in this rapidly growing field. The aim of this review is to (1) introduce the method, (2) give an overview where ECP has shown promising effects and has become an essential part of treatment protocols, and (3) to give recommendations on how to proceed in numerous indications.

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“…Standard therapies include extracorporeal photopheresis, retinoids (bexarotene), histone deacetylase (HDAC) inhibitors (vorinostat and romidepsin), interferon α, methotrexate, pralatrexate, alemtuzumab, brentuximab vedotin, and now, mogamulizumab (Table 1). 4,10,17–29 All have relatively low response rates ranging from 14% to 60% (mostly 20%–30%) and median duration of response rarely exceeding 1 year 10,30…”

Section: Introductionmentioning

confidence: 99%

Mogamulizumab: a new tool for management of cutaneous T-cell lymphoma

Ollila

Şahin

Olszewski

2019

OTT

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Cutaneous T-cell lymphoma (CTCL) poses unique treatment challenges, given its range of presentations and numerous systemic therapy options. These options often lack comparative evidence or are characterized by low response rates and short remission duration in relapsed/refractory disease. The approval of mogamulizumab, a humanized, glycoengineered IgG1κ monoclonal antibody targeting the chemokine receptor type 4 (CCR4) chemokine receptor, brings a novel tool into the spectrum of treatment options for advanced CTCL and adult T-cell leukemia/lymphoma (ATLL). CCR4 is expressed in almost all cases of ATLL, and in a majority of CTCLs, particularly when blood involvement is present. In a Phase III randomized trial, mogamulizumab was associated with 28% overall response rate among patients with relapsed CTCL, median progression-free survival of 7.7 months, and median duration of remission of 14.1 months. Responses are more frequent among patients with Sézary syndrome and within the blood compartment. Common adverse effects include rash and infusion reactions, which are usually low grade. Sentinel reports indicate that exposure to mogamulizumab may result in severe or refractory graft vs host disease after allogeneic bone marrow transplantation, highlighting the need for vigilance and expert management. Further research may establish incremental efficacy of combining mogamulizumab with cytotoxic or immunomodulatory agents in CTCL, ATLL, and possibly other lymphomas and even solid tumors.

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Systemic Treatment Options for Advanced-Stage Mycosis Fungoides and Sézary Syndrome

Cited by 35 publications

References 145 publications

<p>Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL): focus on chlormethine gel</p>

<p>Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL): focus on chlormethine gel</p>

Extracorporeal Photopheresis—An Overview

<p>Mogamulizumab: a new tool for management of cutaneous T-cell lymphoma</p>

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