Mycosis fungoides and Sézary syndrome

Abstract

Mycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sézary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.

Recurrent urticarial plaques in an old man

“…In early stages (IA-IIA), first-line treatment options include topical glucocorticoids, mechlorethamine, phototherapy and localized radiotherapy. Retinoids, total skin electron beam (TSEB) and low-dose methotrexate are second-line therapies [7]. Regarding the treatment and prognosis of this urticarial presentation, we cannot draw conclusions given the scarcity of available information as well as the disparity in these aspects between the two reported cases.…”

Rezidivierende urtikarielle Plaques bei einem alten Mann

“…Die Erkrankung verläuft in drei Phasen und umfasst Flecken, Plaques und Tumore, die sich überschneiden können. Nicht bei jedem Patienten entwickeln sich zwangsläufig alle Phasen [3,7,8]. Die frühen Stadien sind durch erythematöse Flecken und/oder schuppige und atrophische Plaques an sonnengeschützten Stellen gekennzeichnet und haben in der Regel eine günstige Prognose (88 % Fünfjahresüberleben) [2,3,[5][6][7][8].…”

“…Die Behandlung der MF sollte stadienabhängig sein [6,7,11]. In frühen Stadien (IA-IIA) gehören zu den Erstbehandlungsoptionen topische Glukokortikoide, Mechlorethamin, Phototherapie und lokale Strahlentherapie.…”

Care structure of patients with mycosis fungoides and Sézary syndrome in Germany – Care research based on SHI claims data

“…Primary cutaneous lymphomas (PCL) or skin lymphomas are rare skin malignancies in which certain immune cells multiply uncontrolled in the skin. They are part of the heterogeneous group of non-Hodgkin lymphomas (NHL) [1,2]. The incidence of PCL is estimated to be one new case per year per 100,000 population [1,3].…”