Systemic Therapy of Cutaneous T-Cell Lymphomas (Mycosis Fungoides and the Sezary Syndrome)

  title={Systemic Therapy of Cutaneous T-Cell Lymphomas (Mycosis Fungoides and the Sezary Syndrome)},
  author={Pa Bunn and Stephen J. Hoffman and David Norris and Loren E. Golitz and John L. Aeling},
  journal={Annals of Internal Medicine},
Mycosis fungoides and the Sezary syndrome are low-grade, non-Hodgkin lymphomas that have a mature helper T-cell phenotype and that are always associated with cutaneous involvement [1-3]. Although they are often collectively called cutaneous T-cell lymphomas, they must be distinguished from other intermediate- and high-grade non-Hodgkin lymphomas, which may have cutaneous involvement such as peripheral T-cell lymphoma, adult T-cell leukemia-lymphoma, or B-cell non-Hodgkin lymphomas [3]. That… 

Cutaneous T-Cell Lymphomas: Mycosis Fungoides and Sézary Syndrome

Cutaneous lymphomas arising in skin are rare, they can be the cause of significant morbidity and mortality, and in spite of new approved therapies, they remain challenging and uncurable at the current time.

Mycosis fungoides and the Sézary syndrome.

There is no evidence that early aggressive systemic therapy is preferable to conservative therapy in the management of limited disease, and all patients with recalcitrant or extracutaneous disease should be considered for newer investigative therapies.

Mycosis fungoides and Sezary syndrome.

The diagnosis of MF rests on the clinical presentation as well as the histopathologic findings of an epidermotropic lymphoma with light microscopy showing a dermal infiltrate of lymphocytes with hyperconvoluted cerebriform nuclei and Pautrier's microabscesses.

Choosing a systemic treatment for advanced stage cutaneous T-cell lymphoma: mycosis fungoides and Sézary syndrome.

  • M. Duvic
  • Medicine, Biology
    Hematology. American Society of Hematology. Education Program
  • 2015
Mycosis fungoides is the most common variant of cutaneous T-cell lymphomas among the EORTC-ISCL classification and their skin lesions can be well controlled using skin-directed therapies.

Treatment of cutaneous T-cell lymphoma from a dermatologist's perspective.

  • M. Duvic
  • Medicine, Biology
    Clinical lymphoma
  • 2000
New agents including bexarotene (a rexinoid) and DAB(389)IL-2 (interleukin-2 diphtheria fusion protein) offer new therapeutic options that are advantageous for treatment of mycosis fungoides in later stages.

Treatment of Cutaneous T Cell Lymphoma

Over the last decade, extracorporeal photopheresis has been the only single treatment that has been shown to improve survival in patients with Sezary syndrome, although its true efficacy and place in combination therapy remain unclear.

Cutaneous Lymphomas: A Heterogeneous Group of Lymphoproliferative Disorders at Least in Part Sensitive to Interferon Treatment

Cutaneous lymphomas comprise a heterogeneous group of diseases that are characterized by a clonal accumulation of lymphocytes in the skin, and in many cases the histopathologic evaluation of biopsies maybe extended to include immunophenotyping (immunologic analysis of cellular antigen expression using antibodies) or immunogenotyping or immunogenic analysis of antigen receptor genes.

Staging and management of cutaneous T‐cell lymphoma

Treatment of patients with early‐stage disease (IA–IIB) should be limited to skin‐directed therapy and more advanced or resistant disease may be treated with systemic therapies such as extracorporeal photopheresis, immunotherapy, monoclonal antibody therapy, novel retinoids or chemotherapy, and where possible, patients should be entered into clinical trials.

Cutaneous T cell lymphoma

This Primer summarizes the pathophysiology, epidemiology, diagnosis and management of primary cutaneous T cell lymphoma and CTCL, a group of lymphomas that initially manifest in the skin.

Review of Systemic Therapy for Cutaneous T-Cell Lymphomas

The cutaneous T-cell lymphomas (CTCL) mycosis fungoides and Sezary's syndrome are low-grade, non-Hodgkin lymphomas with a mature helper T-cell phenotype that always have a cutaneous component.



Treatment of cutaneous T-cell lymphoma.

The UCSF approach to treatment of CTCL and lymphomatoid papulosis is presented, and controversial issues relating to total skin versus local treatment, prolonged maintenance versus intermittent therapy, and aggressive versus conservative treatment of early stage disease are discussed.

State of the art therapy of mycosis fungoides and Sézary syndrome.

The available treatment options, as well as the approach to the patient with mycosis fungoides or the Sézary syndrome are discussed.

2-Chlorodeoxyadenosine: an active agent in the treatment of cutaneous T-cell lymphoma.

2-CdA is an effective new agent for the treatment of cutaneous T-cell lymphoma and warrants further study both as a single agent and in combination regimens.

Phase II trial of pentostatin in refractory lymphomas and cutaneous T-cell disease.

  • F. CummingsK. Kim M. O’connell
  • Medicine, Biology
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • 1991
It is concluded that dCF is active in refractory lymphomas and CTCLs, should be avoided in patients with a history of serious pulmonary or cardiac diseases, and warrants consideration for incorporation of a low-dosage schedule into conventional combination chemotherapy regimens, including its use with biologic response modifiers.

Clinical and Immunological Evaluation of 5 Cases of Mycosis Fungoides in Advanced Stages

Five patients with mycosis fungoides, hospitalized in the Division of Radiotherapy and Medical Oncology of the Ospedale Civile, Pordenone, from January 1975 to December 1978, were studied and treated as non-Hodgkin lymphomas, revealing a strict correlation between T-lymphocyte function and response to therapy.

Combined modality treatment of cutaneous T cell lymphoma: results of a 6-year follow-up.

It is concluded that combined modality treatment can be safely administered and produces prolonged disease-free survival in some stage I patients, but not in more advanced stage patients.

Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results.

This preliminary study suggests that extracorporeal photochemotherapy is a promising treatment for widespread cutaneous T-cell lymphoma.

Therapy of chronic lymphocytic leukemia and cutaneous T-cell lymphoma with T101 monoclonal antibody.

The findings accompanying the administration of 50 intravenous courses of monoclonal antibody to human T-cell (T101) in eight patients, four with chronic lymphocytic leukemia and four with cutaneous

Evaluation of circulating malignant cells provides prognostic information in cutaneous T cell lymphoma

Detailed review of the blood lymphocyte morphology in patients with diagnosed or suspected CTCL is helpful in predicting extent of disease and prognosis.