Title: Brentuximab vedotin for cutaneous T-cell, CD30-positive, relapsed, refractory Lymphoma [ID1190]
Funder: Department of Health and Social Care

Abstract: To appraise the clinical and cost effectiveness of brentuximab vedotin within its marketing authorisation for treating CD30-positive cutaneous T-cell lymphoma.

Lay abstract: Lymphomas are cancers of the lymphatic system. They are broadly divided into Hodgkin’s and non-Hodgkin’s lymphomas. Cutaneous T-cell lymphoma is a rare type of non-Hodgkin’s lymphoma that affects the skin. It is caused by the uncontrolled growth of T-lymphocytes within the skin. Many types of cutaneous T-cell lymphoma start as flat red patches or plaques on the skin, which progress to skin tumours, and may be itchy and sometimes painful. Some people with cutaneous T-cell lymphoma experience swelling of the lymph nodes. Within the group of cutaneous T-cell lymphoma, distinct subtypes can be distinguished. Not all subtypes of cutaneous T-cell lymphoma express CD30. CD30 expression is present in primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis and together they form the group of primary cutaneous CD30 positive lymphoproliferative disorders. Mycosis fungoides and Sézary syndrome can also express CD30. Sézary syndrome is closely related to mycosis fungoides but cancerous T-cells (called Sézary cells) are also found in the blood. Mycosis fungoides is the most common type of cutaneous T-cell lymphoma and primary cutaneous anaplastic large cell lymphoma is one of the less common subtypes. Between 2009 and 2013, 1,659 people were newly diagnosed with cutaneous T-cell lymphomas of which around 55% were mycosis fungoides and around 10% were primary cutaneous CD30-positive lymphoproliferative disorders (1). The overall incidence of cutaneous T-cell lymphomas in 2013 was around 0.75 per 100,000 (England), and it was more common in men than women (ratio around 1.6:1)(1). The majority of people diagnosed with cutaneous T-cell lymphoma are over the age of 50 but it can also affect young people (1),(2). Treatment options depend on a number of factors, including how much of the skin is involved, nature of the disease (aggressive or slow growing), the type of lesion and whether the cutaneous T-cell lymphoma has spread to lymph nodes or other organs. Current management of cutaneous T-cell lymphoma consists of skin directed therapies and systemic therapies. Skin directed therapies are aimed primarily at the skin and include photo therapy (such as psoralen and ultraviolet A treatment - PUVA, narrow band ultraviolet B treatment); radiotherapy; total skin electron beam therapy (TSEBT); and topical chemotherapy agents, topical corticosteroids and topical retinoids. Systemic therapies are aimed at treating the skin and/or internal organs affected or at risk, and include chemotherapy (such as methotrexate, gemcitabine, liposomal doxorubicin or multi-agent chemotherapy– cyclophosphamide, doxorubicin, vincristine, and prednisone), and immunotherapy (such as bexarotene, interferon alpha, or extracorporeal photopheresis - ECP). Stem cell or bone marrow transplant (such as allogeneic-SCT) may also be a treatment option for some people (for example those with advanced disease, a poor response to systemic therapy, multiple relapses or a short remission). Treatment options for cutaneous T-cell lymphoma can be used either alone or in combination. People may have multiple sequential treatments and remain on maintenance therapy with palliative intent although there is no established standard of care. References 1. Public Health England (2016) Registration of Cutaneous T-Cell Lymphoma (CTCL) in England. National Cancer Registration and Analysis Services Short Report 2016397 2. Abbott, R.A., Aldridge, C., Dojcinov, S. and Piguet, V. (2013), Incidence of primary cutaneous T-cell lymphoma in Wales. Br J Dermatol 169: 1366– 1367

Research activity codes: 6.1 PHARMACEUTICALS
Health categories: CANCER AND NEOPLASMS