Search Database ICD-O-3 Code Lists

Name

Sezary syndrome (SS)

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. Can also present in leukemic form in the peripheral blood or bone marrow.
Involvement of any organ can be seen at advanced stages.

Assign C449 if specific primary site cannot be determined.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Sezary syndrome is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

SS and mycosis fungoides share overlapping clinical and pathological features but are considered separate entities on the basis of differences in clinical behavior and cell of origin

SS is a generalized disease with a leukemic presentation and characteristic involvement of the skin, with redness over most of the body (erythroderma). Lymphadenopathy and the involvement of any visceral organ can be observed at advanced stages, but the most common sites are the oropharynx, lungs, and CNS.

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.

Module Rule

None

Alternate Names

Sezary disease

Definition

Sezary syndrome (SS) is a neoplasm of T lymphocytes, defined by the triad of erythroderma; generalized lymphadenopathy; and the presence of clonally related neoplastic T cells with cerebriform nuclei (Sezary cells) in the skin, lymph nodes, and peripheral blood. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Chromatin modifiers (ARID1A, TRRAP, DNMT3A, TET2)
Driver mutations are also found in DNA damage response pathways (TP53, POT1, ATM), JAK/STAT signalling (STAT5B; JAK3),
Recurrent gain of function mutations (PLCG1, CARD11, CD28, CARMIL2[RLTPR])
UV radiation induced mutations

Immunophenotyping

CD2- (no expression/negative)
CCR4+ (expression/positive)
CCR7+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5- (no expression/negative)
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-10 Codes (Cause of Death codes only)

C84.1 Sezary disease

Corresponding ICD-10-CM Codes (U.S. only)

C84.1_ Sezary disease (effective October 01, 2015)
C84.1A Sezary disease, in remission (effective October 01, 2024)

Signs and Symptoms

Alopecia
Generalized lymphadenopathy
Itchy, pain, peeling and reddened skin
Palmar or plantar hyperkeratoses
Skin: papules, patches, plaques or tumors
Ulcerated skin lesions, which may become infected

Diagnostic Exams

Flow cytometry
Immunophenotyping
Peripheral blood smear
Skin biopsy
T-cell receptor (TCR) gene arrangement test

Progression and Transformation

Increased prevalence of secondary malignancies, both cutaneous and systemic

Epidemiology and Mortality

Incidence: 0.36 cases per 100,000 person years (2-3% of cutaneous T-cell lymphomas)
Age: 60 years median age
Sex: male predominance
Survival: median survival 32 months

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T- and NK-cell neoplasms
Pages: Part B: 674-676

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment. Bethesda, MD: National Cancer Institute. Updated <02/19/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq. Accessed <03/31/2025>. [PMID: 2638928
Section: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq
Glossary