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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Definition

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. 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.

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.

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+
CCR4 expression
CCR7 expression
CD3 positive
CD4 positive
CD5+
CD7 negative
CD8 negative
CD26 negative
Cutaneous lymphocyte antigen (CLA) expression
PD1 (CD279) expression

Treatments

Chemotherapy
Other therapy
Radiation therapy

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 (U.S. only)

C84.1 Sezary disease (effective October 01, 2015)

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

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Mycosis Fungoides (Including S├ęzary Syndrome) Treatment
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq/
Glossary