Multidisciplinary Management of Mycosis Fungoides/Sézary Syndrome

Abstract

Improved technology (T-cell receptor high-throughput sequencing) and increased multicenter collaboration (Cutaneous Lymphoma International Consortium) have led to diagnostic/prognostic advances. Concurrently, numerous genomic studies have enhanced understanding of disease pathogenesis. Advances in SDTs include topical resiquimod, a novel potent Toll-like receptor (TLR) agonist; consensus CTCL phototherapy guidelines; and use of low-dose radiation therapy. Novel systemic therapies for advanced disease of note include targeted antibody drug conjugates (brentuximab vedotin), immune checkpoint inhibitors, and allogeneic hematopoietic stem cell transplantation (HSCT). Our "toolbox" to diagnose and treat the spectrum of MF/SS continues to expand. Further characterization of genomic data going forward will enable a rational approach to selecting and combining therapies to improve patient care.

Mycosis fungoides with a dramatic course in a patient with a history of pyoderma gangrenosum – a case report

“…The greatest problems in differential diagnostics are posed by early-stage forms of MF and erythroderma [7]. The initial symptoms are non-specific and may imitate mild inflammation of the skin or diseases of non-inflammatory aetiology, which can be misleading and delay the diagnosis [3,9,14,24]. In cases of uncertain diagnosis, attention should be paid to the concentrations of total and specific IgE.…”

“…An equally ambiguous parameter mentioned in the literature is the concentration of β2-microglobulin which can be elevated also in autoimmune diseases or kidney failure [7]. In addition to making a correct diagnosis, it is also crucial to precisely assess the stage of the disease, since it determines the choice of treatment which comprises diverse methods [1,9]. Initial stages are treated with skin-targeted therapies, and in advanced stages they are used in combination with systemic treatment [8,9].…”

“…In addition to making a correct diagnosis, it is also crucial to precisely assess the stage of the disease, since it determines the choice of treatment which comprises diverse methods [1,9]. Initial stages are treated with skin-targeted therapies, and in advanced stages they are used in combination with systemic treatment [8,9]. Topical treatment can be based on glucocorticoids, bexarotene, carmustine, mechlorethamine, imiquimod or resiquimod, as nane trzykrotnie, za każdym razem wynik był inny.…”

“…The prognosis varies, depending chiefly on the TNM stage of the disorder and lymphoma subtype [7,8]. Negative prognostic factors include elevated LDH activity, male sex, elderly age, pronounced pruritus, eosinophilia and giant cell transformation [9,10]. Despite that the disease is usually associated with low malignant potential and long survival, up to 25 years [1,7].…”

“…Rokowanie może być zróżnicowane, przede wszystkim w zależności od stopnia zaawansowania choroby wg klasyfikacji TNM, a także podtypu chłoniaka [7,8]. Wśród negatywnych czynników prognostycznych wymienia się: podwyższoną aktywność LDH, płeć męską, zaawansowany wiek, znaczny świąd, eozynofilię oraz transformację olbrzymiokomórkową [9,10]. Mimo to choroba zwykle wiąże się z niewielką złośliwością i wieloletnim przeżyciem, sięgającym 25 lat [1,7].…”