NCI Definition: A generalized peripheral (mature) T-cell neoplasm characterized by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sezary syndrome is an aggressive disease. (WHO, 2001) [1]

Sezary syndromes most frequently harbor alterations in SETD1B, PLCG1, FAS, UBR5, and TP53 [2].

Most Commonly Altered Genes in Sezary Syndrome

SETD1B Loss, PLCG1 S345F, UBR5 R1379W, UBR5 Mutation, and TP53 c.217-c.1178 Missense are the most common alterations in sezary syndrome [2].

Top Alterations in Sezary Syndrome

Significant Genes in Sezary Syndrome


Disease Details

Sezary's Disease, Cutaneous T-cell lymphoma/Sezary syndrome, CTCL / Sezary syndrome
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
OncoTree Name
Sezary Syndrome
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.