Name
Sezary syndrome
ICD-O-1 Morphology
9701/3: Sezary syndrome
Effective
1978 - 1991
ICD-O-2 Morphology
9701/3: Sezary syndrome
Effective
1992 - 2000
ICD-O-3 Morphology
9701/3: Sezary syndrome
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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
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.
Most patients die of opportunistic infections. Prognostic factors include the degree of lymph node and peripheral blood involvement.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Sezary disease
SS
Definition
Sezary syndrome (SS) is a t-cell lymphoma that 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. In additional, one or more of the following criteria are required: an absolute Sezary cell count greater than or equal to 1000 microlitre, an expanded CD4+ T-cell population resulting in a CD4:CD8 ration of greater than or equal to 10, and loss of one or more T-cell antigens.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
DNM3 expression
EPHA4 expression
PLS3 expression
STAT4 underexpression
TP53 inactivation
TR genes are clonally rearranged
TWIST1 expression
Immunophenotyping
CD2+ (expression/positive)
CCR4+ (expression/positive)
CCR7+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD7- (no expression/negative)
CD8- (no expression/negative)
CD26- (no expression/negative)
Cutaneous lymphocyte antigen (CLA)+ (expression/positive)
PD1 (CD279)+ (expression/positive)
Treatments
Chemotherapy
Other therapy
Radiation therapy
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
202.2 Sezary's disease
Corresponding ICD-10 Codes
C84.1 Sezary disease
Corresponding ICD-10-CM Codes (U.S. only)
C84.1 Sezary disease (effective October 01, 2015)
Signs and Symptoms
Alopecia
Generalized lymphadenopathy
Itchy, pain, peeling and reddened skin
Palmar or plantar hyperkeratoses
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
Incidence: 5% of all cutaneous T-cell lymphomas
Sex: male predominance
Survival: 10-20% overall 5 year survival, most patients die of opportunistic infections
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 390-391
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 390-391
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Mycosis Fungoides (Including Sézary Syndrome) Treatment
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq/
Section: General Information About Mycosis Fungoides (Including Sézary Syndrome) Treatment
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq/