Mycosis fungoides and Sézary syndrome: Current challenges in assessment, management and prognostic markers

Abstract

Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires thorough history taking and physical examination, in combination with skin biopsy. In some cases flow cytometry, molecular studies and imaging are also required in order to diagnose and stage the disease. Staging is derived from the tumour-node-metastasis-blood classification and is currently our best attempt to stratify prognosis and hence guide management in this complex disease. Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. Management remains heavily guided by staging, such that patients with early-stage disease generally begin treatment with skin-directed or local therapies and those with advanced-stage disease have many treatment options, including chemotherapy, the use of biological agents, local and total body radiotherapy, as well as haematopoietic stem cell transplantation. Besides staging, many other patient-related factors influence the treatment strategy, particularly where symptom relief is paramount. There are many challenges remaining in the study of Mycosis fungoides and Sézary syndrome and, given the rarity of the disease, concerted worldwide efforts are required to conduct efficient and effective research.

Cyclophosphamide in dermatology

“…20,91 Cyclophosphamide monotherapy was previously used in the treatment of mycosis fungoides, but multiagent regimens such as CHOP are used today. 21 The combination of cyclophosphamide and fludarabine chemotherapy has also been recommended as a secondline treatment for refractory or progressive Sézary syndrome, based on a prospective study of eight patients.…”

Chronic pruritic dermatitis and peripheral eosinophilia in a 42-year-old man

“…Although an unequivocal diagnosis of SS was established in this patient, it was still difficult to exclude underlying allergic reactions to various medications at various times during his course that may have exacerbated his dermatologic symptoms and contributed to the hypereosinophilia. 13 Treatment of SS is guided by accurate staging 14 and typically involves multimodality immunomodulatory combination therapy, including skin-directed treatment (either nitrogen mustard ointment, phototherapy, or total skin electron beam RT), plus interferon alfa 2b, plus bexarotene, and monthly extracorporeal photopheresis. Response is expected to take 3-18 months.…”

A case report of the long treatment experience of a Sézary syndrome responder patient: 16 years through all the systemic and innovative therapies

“…The majority of available therapies for MF/SS are not able to induce prolonged remissions or survivals: patients should be offered treatment only when required in order to reduce toxicity, following a stage‐adapted approach. In fact, the main purposes of therapies are palliation and improvement of quality of life . Moreover, despite the availability of various treatment strategies, including novel biological drugs, disease control remains challenging and often requires multiple lines of therapy …”

“…In fact, the main purposes of therapies are palliation and improvement of quality of life . Moreover, despite the availability of various treatment strategies, including novel biological drugs, disease control remains challenging and often requires multiple lines of therapy …”

“…In fact, the main purposes of therapies are palliation and improvement of quality of life. 2,3 Moreover, despite the availability of various treatment strategies, including novel biological drugs, disease control remains challenging and often requires multiple lines of therapy. [1][2][3][4] In particular, patients with SS with or without the typical generalized erythroderma have a poor prognosis (median survival, 4 y).…”