The Pathogenesis of Mycosis Fungoides

EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome

“…7 Patches and plaques may also exhibit hypopigmentation or hyperpigmentation. 1 Plaques can be followed by tumours, although it is common for patients to have patch, plaque and tumour lesions simultaneously on different parts of the body. 7 The from patches to plaques to tumours is variable and unpredictable.…”

“…Tumours are the presenting stage in about 10% of cases and are characterised by protruding, often ulcerative, lesions and lymphocytic infiltration into the dermis. 1,7 Infection, secondary to ulceration, is a frequent cause of morbidity. 3 Some patients with patch-stage MF will never progress to other forms of the disease, but many will eventually develop advanced disease.…”

“…7 Patients in whom erythema extends to 80% or more of the total skin surface are significantly more likely to have leukaemic involvement in the peripheral blood. 1 SS describes patients with generalised erythroderma and socalled Sé zary cells in the peripheral blood. 1,3 These are enlarged, atypical lymphocytes with characteristic convoluted nuclei, which can be found in the peripheral blood of approximately 25% and 10% of patients with cutaneous tumours and generalised plaques, respectively.…”

“…Approximately two-thirds of these lymphomas are of T-cell origin, and the pathogenesis and management of both T-and B-cell cutaneous lymphomas have been recently reviewed. [1][2][3][4][5][6] The most common form of cutaneous T-cell lymphoma (CTCL) is mycosis fungoides (MF), accounting for around 60% of new cases. Sé zary syndrome (SS) is much rarer and accounts for only 5% of CTCL cases.…”

“…7,13 The choice of treatment is often determined by physician or patient preference, or institutional experience, particularly as there is a paucity of data from phase III trials and a lack of consensus concerning treatment for later stages of MF/SS. 3,[14][15][16] However, a number of authors have published recommendations or reviews on the management of CTCL, 1,3,7,15,17 , and guidelines have been published jointly by the British Association of Dermatologists and the UK Cutaneous Lymphoma Group. 16 Nonetheless, treatment choices vary across Europe and there are, as yet, no uniform European guidelines for the management of CTCL.…”