Case 119 – Summary

 Many thanks for all your great suggestions and contributions this week. We highlighted the multidisciplinary treatment of our patient with mycosis fungoides, who eventually required systemic treatment with brentuximab vedotin for his CD30+ disease.

Primary cutaneous T cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma (NHL) which presents in the skin. It is the second most common group of extranodal NHL but remains a rare disorder, with an estimated annual incidence of 1 per 100,000 of the population in the UK, with a higher male incidence (1.6:1.0) and peak age between 50 and 74. Mycosis Fungoides (MF) is the most common subtype of CTCL, comprising around 40% of these conditions. Other less common subtypes include primary cutaneous anaplastic large cell lymphoma (pcALCL) and Sezary syndrome.

Patients with MF at different clinical stages present with skin patches/plaques, tumours or erythroderma; accurate staging relies on clinical, histological, immunophenotypical and genetic data.

All patients with suspected or proven primary cutaneous lymphomas should be reviewed at a regional Specialist Skin Cancer Multidisciplinary Team (MDT) meeting, with close involvement of haematology MDT in the presence of systemic disease. The current staging system in use in the UK is the International Society for Cutaneous Lymphomas (ISCL) EORTC revised staging system. This system distinguishes patches and plaques, incorporates a molecular assessment of lymph node and peripheral blood, and provides a quantitative method for assessing peripheral blood disease in SS (see links at bottom).

Treatment options will often be dictated by the clinical stage of disease. Options include skin-directed therapies (SDT) such as topical agents, phototherapy and radiotherapy, and systemic therapies including biologics, chemotherapy and stem-cell transplantation.

Early stage MF (Stage IA – IIA) is often treated with skin-directed therapy including topical steroids and phototherapy. Patients with infiltrative plaques or tumours (stage IIB) may also be offered local radiotherapy.  Those with more systemic disease may be offered skin-directed therapies in conjunction with systemic biologic therapies e.g. interferon alfa or retinoids. Total skin electron beam therapy has also been used in this group. Those with advanced and refractory disease often require antibody therapy or chemotherapy. Allogeneic stem cell transplant can also be considered.

Extra reading:

ESMO clinical practice guideline: https://www.esmo.org/Guidelines/Haematological-Malignancies/Primary-Cutaneous-Lymphoma

British Association of Dermatologists guideline: http://www.bad.org.uk/shared/get-file.ashx?id=6265&itemtype=document

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