Lymphoma & related disorders
Mature T/NK cell disorders
T/NK cell disorders with a leukemic component
Sezary syndrome
Author: Dragos C. Luca, M.D.
Topic Completed: 1 August 2011
Minor changes: 16 March 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Sézary syndrome [title]
Table of Contents
Definition / general | Epidemiology | Sites | Etiology | Clinical features | Case reports | Prognosis and treatment | Postulated normal counterpart | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Luca D. Sezary syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBsezary.html. Accessed November 23rd, 2020.
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
- 63 year old man with thrombotic microangiopathy (Nephrol Dial Transplant 2004;19:733)
- 66 year old man without erythroderma (Indian J Dermatol Venereol Leprol 2002;68:225)
- 74 year old man with coexisting CLL (Ann Biol Clin (Paris) 2003;61:94)
- 75 year old man with large cell transformation (Am J Clin Pathol 2000;113:792)
Prognosis and treatment
- Poor prognosis; better with categories LN 0 - 2 or Dutch classification I - II with negative T cell rearrangement by PCR
- 10 - 20% 5 year survival rate; most die of opportunistic infections
- May transform to anaplastic large cell lymphoma with poor prognosis
- Staging: ISCL/EORTC Revision to the classification of mycosis fungoides and Sézary syndrome
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
Contributed by Dr. Mark R. Wick
Skin
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
- Chronic actinic reticuloid
- Drug induced erythroderma
- Eczema and psoriasis for cases with nonspecific histology
