Successful treatment of Sezary syndrome with extracorporeal photopheresis - The first attempt in India.

Abstract

Sezary syndrome (SS) is more aggressive leukemic variant of cutaneous T-cell lymphoma in which a significant number of circulating malignant (Sezary) cells are observed in peripheral blood. Although single-agent or combination chemotherapy regimens have produced moderately high response rates in patients with advanced-stage SS, these responses are invariably not durable. Extracorporeal photopheresis (ECP) is recommended as an immunomodulator treatment, offering better life quality for patient. We would like to present the first SS case treated successfully with low-dose methotrexate and ECP in India. A 50-year-old male presented with rash and severe pruritus all over the body for 2 years. He had received various treatment regimens but without any symptomatic improvement. He underwent detailed examination and diagnosis of SS was established. Peripheral smear revealed total leukocyte count of 14900/μl with 55% cells reported as Sezary cells. Contrast-enhanced computerized tomography revealed few insignificant (<1.5 cm) bilateral nodes in the axillary and inguinal region. The patient's disease stage was determined IVA1, and grade was T4N0M0B2. He received six cycles of CHOP, which led to a short-term remission of <3 months, and he was started on single-agent methotrexate along with skin supportive treatment. He did not respond to low-dose methotrexate alone, and therefore, ECP was added to treatment regimen. This was possibly the first such treatment for SS patient in India. The patient had very good response after six cycles of ECP with pruritus and itching diminishing and scaly lesions down to <10% of body surface area. There was regrowth of hair all over affected area. Sezary cell counts also came down to 35%. The patient continues to do well post-ECP, with single-agent gemcitabine. ECP either as monotherapy or in combination with other immunotherapies offers a good treatment option to otherwise resistant cases of SS.