Staging and prognosis of mycosis fungoides and Sézary syndrome
- Richard T Hoppe, MD
Richard T Hoppe, MD
- Professor of Radiation Oncology
- Stanford University School of Medicine
- Youn H Kim, MD
Youn H Kim, MD
- Professor of Dermatology
- Stanford University School of Medicine
- Section Editors
- Timothy Michael Kuzel, MD, FACP
Timothy Michael Kuzel, MD, FACP
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine, Rush University Medical Center
- Chief, Division of Hematology/Oncology/Cell Therapy
- John A Zic, MD
John A Zic, MD
- Section Editor — Cutaneous Lymphoma
- Associate Professor of Medicine/Dermatology
- Vanderbilt University School of Medicine
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T cell lymphoma (CTCL).
●MF is a mature T cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Skin lesions include patches or plaques that may be localized or widespread, tumors, and erythroderma.
●SS is a distinctive erythrodermic CTCL with a leukemic involvement of malignant T cells clonally matching that in the skin.
This group of disorders differs from other primary CTCLs by virtue of unique clinical features and histopathology. This topic review will discuss the staging, response criteria, and prognosis of MF and SS. The diagnosis and management of these disorders are discussed separately. (See "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides" and "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome" and "Treatment of early stage (IA to IIA) mycosis fungoides" and "Treatment of advanced stage (IIB to IV) mycosis fungoides".)
TNMB criteria — The standard staging classification system for mycosis fungoides (MF) and Sézary syndrome (SS) is the TNMB system, which is based upon an evaluation of the skin (T), lymph nodes (N), visceral involvement (M), and blood (B) (table 1A-B) . The standard staging evaluation includes a careful examination of the skin (especially the scalp, palms, soles, and perineum) with skin biopsy, a complete blood count with Sézary cell analysis, screening chemistries including lactate dehydrogenase, and a chest x-ray (table 2). Lymph node biopsies, not fine needle aspirations, are obtained if lymphadenopathy is present, since lymph node involvement affects both the stage and prognosis.
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- TNMB criteria
- Skin evaluation
- - Total body skin examination
- - Percent body surface area estimates
- - Disease severity estimates
- Peripheral blood
- Imaging studies
- Lymph node biopsy
- Bone marrow aspirate and biopsy
- Impact of disease stage
- Folliculotropic variant
- Transformation to large cell histology
- Younger age
- Prognostic indices
- RESPONSE ASSESSMENT
- RESPONSE CRITERIA
- SUMMARY AND RECOMMENDATIONS