Sezary Syndrome

Sezary syndrome and mycosis fungoides are the most common forms of cutaneous T-cell lymphoma (CTCL) and can mimic benign skin disorders. This can be a diagnostic challenge to clinicians. Though mycosis fungoides is the most common cutaneous lymphoma, it constitutes less than 1% of non-Hodgkin lymphoma cases. Researchers hypothesize that Sezary syndrome can evolve gradually from mycosis fungoides or occur spontaneously. Significant evidence has been established that Sezary syndrome and mycosis fungoides are closely related and constitute the broad spectrum of cutaneous lymphomas.

Primary cutaneous lymphomas (PCL) are localized to the skin without extracutaneous involvement at the initial diagnosis and are a subset of non-Hodgkin lymphoma. They can originate from T or B lymphocytes called cutaneous T-cell lymphomas or cutaneous B-cell lymphomas (CBCL), respectively. Cutaneous T-cell lymphoma is further categorized into 2 types:

1. An indolent form that includes mycosis fungoides, lymphomatoid papulosis, and anaplastic large T-cell primary cutaneous lymphoma

2. An aggressive form that includes Sezary syndrome