Oral bexarotene in a therapy‐resistant Sézary syndrome patient: observations on Sézary cell compartmentalization

Abstract

Background A 63-year-old man with therapy-resistant Sézary syndrome was enrolled in a multicenter trial of oral bexarotene for advanced-stage cutaneous T-cell lymphoma (CTCL).Methods Monthly evaluations for efficacy and side-effects were conducted and documented.Results Gradual improvement in erythema, pruritus, and scale was noted during the initial 16-week trial period and treatment was extended to 40 weeks. From week 20 to week 40, the erythroderma continued to improve and the lymph node burden decreased, but the absolute Sézary cell count inversely increased. By week 40, intractable pruritus and erythroderma abruptly recurred, and bexarotene was discontinued.Conclusions Bexarotene is well tolerated and can be efficacious in patients with Sézary syndrome. Shifting of Sézary cells between different compartments was noted. Further studies on the interaction between the skin, lymph nodes, and peripheral blood compartments during bexarotene treatment in this subset of patients with CTCL are needed.

Bexarotene treatment of late-stage mycosis fungoides and Sézary syndrome: Development of extracutaneous lymphoma in 6 patients∗

“…A report about this patient (patient 5 of the present study) has been published. 4 We observed similar changes in other patients in whom a lower dose of bexarotene was combined with extracorporeal photopheresis (ECP). In these patients, clinical improvement in skin signs and symptoms was associated with development of solid organ lymphoma.…”

Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

“…93,101,113,114,120 Also noted has been an improvement in the skin with bexarotene treatment while either no change or worsening of the blood tumor burden has occurred. 91,121,122 Publications using bexarotene in combination with other therapies in the treatment of SS are shown in Table II. 91,93,95,96,100,101,113,115 The two most common side effects of bexarotene include hyperlipidemia and central (secondary) hypothyroidism.…”

Extracutaneous Tumour Mass Following Bexarotene and Interferon Therapy of Primary Cutaneous CD30+ Large Cell Lymphoma

“…One might speculate that the beneficial effects are restricted to cutaneous T-cell infiltration, whereas circulating tumour cells are relatively unaffected (7). Furthermore bexarotene has been shown to down-regulate E-selectin on endothelial cells, thus causing the entrapment of cutaneous homing T lymphocytes in the circulation (8).…”