Cutaneous T-cell Lymphoma
Section snippets
Staging and prognosis of mycosis fungoides/SÉzary syndrome
Staging systems for MF have been developed based on clinical features of skin involvement as well as infiltration of lymph nodes and viscera. The most commonly used staging system for MF/SS is based on a tumor (T)-node (N)-metastasis (M)-blood (B) classification (Table 2).15 Skin involvement is defined by the type and extent of lesion. T1 and T2 disease are patches or plaques involving less than or more than 10% of the skin surface, respectively. T3 disease is the presence of at least one
Treatment
The diagnosis and initial treatment of CTCL usually takes place in the dermatologist's office. Because of the panoply of therapies available and the chronic progressive clinical course in most patients, management of the disease ideally involves a multidisciplinary team incorporating expertise in skin-directed therapy, radiation therapy, photopheresis, infusional and oral anti-neoplastic therapies, and combined-modality treatment. The therapeutic decision should incorporate the disease stage,
Summary
Although a number of treatments have shown efficacy in the symptomatic management of CTCL, cure remains elusive for most patients. The overall goals of treatment for most patients are to provide palliation for symptoms of pruritus and compromised skin integument and to prevent further immunosuppression. Given the low overall incidence of CTCL, the disease chronicity, the morbidity, and the lack of effective cure, enrollment in clinical trials should be encouraged so that more effective agents
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Cited by (30)
Cutaneous T Cell Lymphoma: A Difficult Diagnosis Demystified
2019, Dermatologic ClinicsCitation Excerpt :The combination of methotrexate with this treatment modality increases the overall efficacy by inhibiting the inactivation of the FAS promoter.90 Allogeneic stem cell transplantation has been shown to be highly efficacious for the treatment of CTCL, providing a sustained immune-mediated graft-versus-lymphoma effect.11 Although morbidity and mortality after this therapy is relatively high, possibly given the advanced age of CTCL patients and the immunosuppression typical of the disease and its treatment, stem cell transplantation can provide long-term remission in a subset of patients.91
Romidepsin for the treatment of relapsed/refractory cutaneous T-cell lymphoma (mycosis fungoides/Sézary syndrome): Use in a community setting
2016, Critical Reviews in Oncology/HematologyCitation Excerpt :Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of non-Hodgkin lymphomas (NHL) in which malignant, mature, post-thymic T cells initially arise in the skin (Lansigan et al., 2008).
Sézary syndrome: A study of 176 patients at Mayo Clinic
2012, Journal of the American Academy of DermatologyCitation Excerpt :Another prognostic factor was the presence of TCR gene rearrangements in skin and blood. In the literature, the prognostic factors associated with worse outcomes include advanced age, enlargement of peripheral lymph nodes, increased leukemic burden in the blood, increased LDH levels, low percentage of CD8+ cells in lymph nodes, and large-cell transformation.2,3,8,10,24,35 Because of a lack of data on the disease course of many patients, we were not able to assess for certain prognostic factors, such as large-cell transformation.
Mycosis fungoides with testicular involvement: A rare phenomenon
2012, Leukemia ResearchPocket Guide to Diagnostic Hematopathology
2019, Pocket Guide to Diagnostic Hematopathology