Maxim Polansky1, Rakhshandra Talpur2, Seema Daulat3, Chitra Hosing4, Bouthaina Dabaja5, Madeleine Duvic3. 1. Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX; University of Texas at Houston Medical School, Houston, TX. 2. Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address: rtalpur@mdanderson.org. 3. Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX. 4. Department of Stem Cell and Transplantation, University of Texas MD Anderson Cancer Center, Houston, TX. 5. Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, TX.
Abstract
INTRODUCTION: Sézary syndrome (SS) is a rare leukemic variant of cutaneous T-cell lymphoma (CTCL). It presents with 80% erythroderma of the body, the presence of > 1000 Sézary cells in the peripheral blood, lymphadenopathy, and pruritus. Complete remission or response (CR) is rare in patients with SS. PATIENTS AND METHODS: An analysis of a prospective database identified 217 patients with SS (14%), whose long-term CRs (> 1 years) are reported. RESULTS: Of 217 patients with SS, 18 (8.3%) achieved a CR (14 Caucasians, 2 African Americans, and 2 Hispanics; 9 women and 9 men). The median age at diagnosis was 53.5 years (range, 21-80 years). The stage at the initial diagnosis was IB in 1, III in 2, IVA in 4, and IVB in 11. The median duration of CR was 6.25 years (range, 1-13 years). The average interval between the diagnosis and CR was 2 years (range, 1-11 years). Three CRs were achieved with immunomodulatory therapy (extracorporeal photopheresis, interferon-α, and/or retinoids), antibiotics, and topical steroids. One CR was achieved with alemtuzumab and another with mogamulizumab. The other 13 CRs were achieved after allogeneic stem cell transplantation (SCT). Seven patients achieved a durable response (DR) with a 5.1-year disease-free interval. Three patients with a DR after SCT died of sepsis after 2 years with relapse. Of the remaining 4 patients, 3 achieved a DR from medical therapy alone and were alive with stable disease at the last follow-up visit. The fourth patient died of sepsis 1 year after relapse. CONCLUSION: SS is a rare and aggressive type of CTCL that is refractory to current therapies. We report that CRs can be achieved in 3 methods: combined immunomodulatory therapy, targeted biologic agents, or total skin electron beams followed by nonablative allogeneic SCT.
INTRODUCTION: Sézary syndrome (SS) is a rare leukemic variant of cutaneous T-cell lymphoma (CTCL). It presents with 80% erythroderma of the body, the presence of > 1000 Sézary cells in the peripheral blood, lymphadenopathy, and pruritus. Complete remission or response (CR) is rare in patients with SS. PATIENTS AND METHODS: An analysis of a prospective database identified 217 patients with SS (14%), whose long-term CRs (> 1 years) are reported. RESULTS: Of 217 patients with SS, 18 (8.3%) achieved a CR (14 Caucasians, 2 African Americans, and 2 Hispanics; 9 women and 9 men). The median age at diagnosis was 53.5 years (range, 21-80 years). The stage at the initial diagnosis was IB in 1, III in 2, IVA in 4, and IVB in 11. The median duration of CR was 6.25 years (range, 1-13 years). The average interval between the diagnosis and CR was 2 years (range, 1-11 years). Three CRs were achieved with immunomodulatory therapy (extracorporeal photopheresis, interferon-α, and/or retinoids), antibiotics, and topical steroids. One CR was achieved with alemtuzumab and another with mogamulizumab. The other 13 CRs were achieved after allogeneic stem cell transplantation (SCT). Seven patients achieved a durable response (DR) with a 5.1-year disease-free interval. Three patients with a DR after SCT died of sepsis after 2 years with relapse. Of the remaining 4 patients, 3 achieved a DR from medical therapy alone and were alive with stable disease at the last follow-up visit. The fourth patient died of sepsis 1 year after relapse. CONCLUSION: SS is a rare and aggressive type of CTCL that is refractory to current therapies. We report that CRs can be achieved in 3 methods: combined immunomodulatory therapy, targeted biologic agents, or total skin electron beams followed by nonablative allogeneic SCT.
Authors: M Kinoshita-Ise; T Ouchi; E Izumi; O Kawaguchi; K Nagao; M Amagai; T Funakoshi Journal: Clin Exp Dermatol Date: 2017-12-21 Impact factor: 3.470
Authors: C Hosing; R Bassett; B Dabaja; R Talpur; A Alousi; S Ciurea; U Popat; M Qazilbash; E J Shpall; Y Oki; Y Nieto; C Pinnix; M Fanale; F Maadani; M Donato; R Champlin; M Duvic Journal: Ann Oncol Date: 2015-09-28 Impact factor: 32.976