Sezary syndrome

Name
Sezary syndrome
ICD-O-1 Morphology
9701/3
Effective 1978 - 1991
ICD-O-2 Morphology
9701/3
Effective 1992 - 2000
ICD-O-3 Morphology
9701/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
Cutaneous (skin) lymphoma which presents with generalized skin lesions. See Module 7.

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Grade
5 - T-cell
Module Rule
None
Alternate Names
Definition
Sezary syndrome is a generalized mature T-cell leukemia characterized by the presence of erythroderma, lymphadenopathy and neoplastic T-lymphocytes in the blood. The neoplastic T-cells have cerebriform nuclei, and the disease is by tradition regarded as a variant of Mycosis Fungoides. However, the behavior is usually much more aggressive.
Abstractor Notes
Sezary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy, and the presence of clonally related neoplastic T-cells with cerebriform nuclei (Sezary cells) in skin, lymph nodes, and peripheral blood.

SS is a leukemia and thus, by definition, a generalized disease. All visceral organs may be involved in the terminal stages, however, there is often a remarkable sparing of the bone marrow. This is an aggressive disease with an overall survival rate at 5 years of 10-20%.

Most patients die of opportunistic infections. Prognostic factors include the degree of lymph node and peripheral blood involvement.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Amplification JUNB
Inactivation TP53
T-cell receptor genes clonally rearranged
Translocation and associated deletions 1p, 6q
Immunophenotyping
CD2+
CD3+
CD4+
CD5+
CD7-
CD26-
Express CCR4
Express CLA
TCRB+
Treatments
Chemotherapy
Other RX
Radiation
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
202.2 Sezary's disease
Corresponding ICD-10 Codes
C84.1 Sezary's disease
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C84.1 Sezary disease
Signs and Symptoms
Itchy, pain, peeling and reddened skin
Palmar or plantar hyperkeratoses
Skin: papules, patches, plaques or tumors
Ulcerated skin lesions, which may become infected
Progression and Transformation
Increased prevalence of secondary malignancies, both cutaneous and systemic
Epidemiology and Mortality
Age: 60 years median age
Sex: male predominance
Survival: 10-20% overall 5 year survival, most patients die of opportunistic infections