Mycosis fungoides

Question 1

Mycosis fungoides background

Answer 1

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T cell lymphoma

MF is a mature T cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Skin lesions include patches or plaques that may be localized or widespread, tumors, and erythroderma

Question 2

early stage

Answer 2

1 - plaques less than 10% of skin surface
2 - plaques of any size and/or lymph node involvement

treat with topical therapies

●Topical corticosteroids
●Topical chemotherapy (nitrogen mustard or carmustine)
●Topical retinoids
●Local radiation (x-ray or electron beam)
●Phototherapy (UVB or PUVA)

Question 3

Late stage

Answer 3

For patients with generalized tumors, total skin electron beam therapy (TSEBT) or systemic therapies are acceptable treatment options.

For patients with lymph node or visceral disease, we suggest treatment with romidepsin or systemic chemotherapy

Question 4

Romidepsin

Answer 4

Romidepsin, is an anticancer agent used in cutaneous T-cell lymphoma

thiol prodrug
The thiol reversibly interacts with a zinc atom in the binding pocket of Zn-dependent histone deacetylase to block its activity. Thus it is an HDAC inhibitor.