Clinical presentation, pathologic features, and diagnosis of Sézary syndrome
- Alain H Rook, MD
Alain H Rook, MD
- Professor of Dermatology
- Perelman School of Medicine at the University of Pennsylvania
- Elise A Olsen, MD
Elise A Olsen, MD
- Professor of Dermatology and Oncology
- Duke University Medical Center
- 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
Sézary syndrome (SS) and mycosis fungoides (MF) 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, plaques, or tumors that may be localized or widespread and erythroderma.
●SS is defined as a distinctive erythrodermic CTCL with a leukemic involvement of malignant T cells clonally matching that in the skin.
SS may be considered to have either evolved from MF (ie, not initially meeting the criteria for SS), or may present de novo with full blown signs and symptoms of the condition . In either case, both MF and SS are defined histologically and staged by the same criteria . In contrast to patch/plaque MF, SS is much more symptomatic, has a lower potential for remission, and lower expected survival.
In 2007, the staging system for MF and SS was revised to incorporate blood involvement and a standardized definition of erythroderma . In 2011, further revisions to the blood and lymph node staging were published that further define and standardize the extracutaneous extent of the disease . These changes in staging permit one to assess the outcomes in patients with erythrodermic MF separately from those of SS.
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- CLINICAL MANIFESTATIONS
- - Skin lesions
- - Lymphadenopathy
- - Viscera
- - Pruritus
- Associated conditions
- - Infections
- - Second malignancies
- PATHOLOGIC FEATURES
- Skin biopsy
- - Light microscopic findings
- - Clonality of the T cell receptor (TCR) gene rearrangement
- - Immunophenotyping confirming T cell origin (CD3+, CD4+)
- Lymph node biopsy
- Peripheral blood
- - Morphology
- - Flow cytometry
- - TCR gene rearrangement clonality
- Site of biopsy
- Diagnostic criteria
- DIFFERENTIAL DIAGNOSIS
- Mycosis fungoides
- Primary skin disorders
- - Psoriasis
- - Pityriasis rubra pilaris
- - Atopic dermatitis
- - Contact dermatitis
- - Chronic actinic dermatitis
- - Scabies
- - Drug eruption
- - Graft-versus-host disease
- - Idiopathic
- Other lymphoproliferative or hematologic diseases
- - Adult T cell leukemia lymphoma
- - T cell prolymphocytic leukemia
- - Hypereosinophilic syndrome
- SUMMARY AND RECOMMENDATIONS