Lymphoma and plasma cell neoplasms
T / NK cell disorders
Sézary syndrome



Topic Completed: 1 August 2011

Revised: 7 February 2019

Copyright: 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Sézary syndrome [title]


Dragos C. Luca, M.D.
Page views in 2018: 3,098
Page views in 2019 to date: 3,492
Cite this page: Luca D. Sezary syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBsezary.html. Accessed November 18th, 2019.
Definition / general
  • Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and the presence of clonally related neoplastic T cells with cerebriform nuclei (Sézary cells) in skin, lymph nodes and peripheral blood
  • In addition, one or more of the following criteria are required: an absolute Sézary cell count of at least 1000/mm³, an expanded CD4+ T cell population resulting in a CD4 / CD8 ratio of more than 10 and / or loss of one or more T cell antigens (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)
Epidemiology
  • Rare, < 5% of all cutaneous T cell lymphomas
  • Adults, usually men over 60
Sites
  • By defition, a leukemia, therefore a generalized disease
  • All visceral organs may be involved in the terminal stages
  • Remarkable sparing of the bone marrow
Etiology
  • Unknown but a syndrome clinically indistinguishable is occasionally seen in HTLV-1 associated leukemia/lymphoma
Clinical features
  • Generalized exfoliative erythroderma, generalized lymphadenopathy and blood involvement by Sézary cells (resemble cells of mycosis fungoides)
  • Pruritus, alopecia, ectropion, palmar / plantar hyperkeratosis, onychodystrophy, leonine facies (resembles a lion)
  • Associated with monoclonal gammopathy and secondary neoplasms (carcinoma, other non-Hodgkin lymphomas), attributed to the loss of normal circulating CD4+ cells
  • Skin lesions rarely become tumor masses
Case reports
Prognosis and treatment
Postulated normal counterpart
  • Mature epidermotropic skin homing CD4+ T cells
Clinical images

Images hosted on other servers:

Erythroderma

Gross description
  • Erythroderma, generalized lymphadenopathy, rarely skin tumors
Microscopic (histologic) description
  • Atypical lymphocytes (Sézary cells) are intermediate / large with basophilic cytoplasm, cerebriform nuclei, inconspicuous nucleoli; marrow usually normal, if involved the infiltrates are sparse and mainly interstitial
  • May resemble mycosis fungoides in tissue but more often monotonous infiltrates and sometimes no epidermotropism
  • 1/3 of cases have nonspecific histology of the skin biopsy
  • Peripheral blood: Romanowsky stained smears show large cells with slight cytoplasm containing PAS+, perinuclear vacuoles and large, cerebriform, hyperchromatic nuclei
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Dr. Mark R. Wick

Skin

Positive stains
Negative stains
Molecular / cytogenetics description
  • Clonal rearrangement of T cell receptor genes but no specific chromosomal abnormalities
  • Complex karyotypes including abnormalities of 1p, 6q, 10q, 17p, 19
  • TP53 and p16 inactivation, JUNB amplification
Differential diagnosis
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