From the Guidelines
Sézary syndrome should be treated with extracorporeal photopheresis (ECP) as the first-line treatment, which can be combined with other modalities such as interferon-alpha or low-dose methotrexate, to improve overall response rates and quality of life. This approach is based on the most recent and highest quality study available, which suggests that ECP is a effective treatment for Sézary syndrome, with overall response rates ranging from 30% to 80% and complete response rates ranging from 14% to 25% 1.
Treatment Options
- ECP: removes and treats blood cells with ultraviolet light before returning them to the patient, performed on two consecutive days every 2-4 weeks
- Interferon-alpha: 3-9 million units three times weekly
- Low-dose methotrexate: 5-25 mg weekly
- Skin-directed therapies: topical corticosteroids, nitrogen mustard, or PUVA (psoralen plus ultraviolet A) therapy for symptom management
- Targeted therapies: bexarotene (300 mg/m² daily), mogamulizumab, or alemtuzumab for more advanced disease
- Histone deacetylase inhibitors: vorinostat or romidepsin as additional options
- Allogeneic stem cell transplantation: may be considered for eligible patients with advanced disease
Disease Characteristics
- Sézary syndrome is a rare and aggressive type of cutaneous T-cell lymphoma
- Characterized by the presence of malignant T-cells in the skin, lymph nodes, and blood
- Leads to erythroderma (reddening of the skin), intense itching, and potential immunosuppression
- Requires ongoing monitoring of blood counts, skin condition, and lymph node status
Evidence-Based Recommendations
The recommendation to use ECP as the first-line treatment for Sézary syndrome is based on the most recent and highest quality study available, which was published in 2018 1. This study suggests that ECP is a effective treatment for Sézary syndrome, with overall response rates ranging from 30% to 80% and complete response rates ranging from 14% to 25%. Other studies, such as those published in 2013 1, 2009 1, and 2011 1, also support the use of ECP as a treatment for Sézary syndrome. However, the 2018 study is the most recent and highest quality study available, and its findings should be given the most weight in guiding treatment decisions. Additionally, a 2019 study published in the British Journal of Dermatology also supports the use of ECP as a treatment for Sézary syndrome, and provides guidance on the management of primary cutaneous lymphomas 1.
From the Research
Definition and Characteristics of Sezary Syndrome
- Sezary syndrome is a rare and aggressive variant of cutaneous T-cell lymphoma characterized by erythroderma, generalized lymphadenopathy, and atypical lymphocytes in peripheral blood 2.
- It is the leukemic form of primary cutaneous T-cell lymphoma, with the lowest reported median survival of all cutaneous lymphomas 3.
- Patients with Sezary syndrome often experience symptoms such as pruritus, palmo-plantar hyperkeratosis, nail lesions, alopecia, and ectropion 2.
Treatment Options for Sezary Syndrome
- Novel systemic therapies, including interferon alfa, bexarotene, histone deacetylase inhibitors, brentuximab vedotin, and mogamulizumab, are generally prescribed to patients with advanced-stage disease or those with early-stage disease refractory to skin-directed therapies 4.
- Extracorporeal photopheresis is particularly effective for patients with Sezary syndrome, with an overall response rate of 62% in one study 5.
- Allogeneic transplantation is becoming increasingly used for younger patients 4.
- A three-line treatment approach, including extracorporeal photopheresis, immunomodulators, and photo-photochemotherapy, has been used as a first-line option for induction of remission 2.
Prognostic Factors and Survival
- Prognostic variables, such as hypereosinophilia, highly elevated β2µglobulin, male sex, and highly elevated LDH, have been identified as significant factors in Sezary syndrome 2.
- The overall survival after diagnosis is approximately 2.6 years, with a median time to response of 10 months 2, 5.
- The 2- and 4-year predicted overall survivals have been reported to be 82% in one study 5.
- Improved comprehension of Sezary syndrome pathogenesis is progressively increasing the still poor survival, with a reported overall survival of 38.5 months in patients followed in the last five years 2.