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

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

“…There have been several reports, primarily in patients with SS, of acute worsening or more aggressive disease (large cell transformation, extracutaneous disease, CD81 lymphoma) after starting bexarotene: the causality is not clear in these cases. 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.…”

“…111 The patients with SS treated with bexarotene monotherapy are reported in Table I. [110][111][112][113][114] Tsirigotis et al 115 noted a 100% RR in 3 patients with MF and two with SS treated with a combination of ECP and bexarotene, and Ranki 91 noted a PR in one patient with SS treated with a combination of interferon alfa and bexarotene. Richardson et al 92 noted an 89% RR rate in 28 patients treated with ECP, interferon alfa, andein 24 of 28 casesebexarotene.…”

“…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. 109 In patients taking 300 mg/m 2 /d or more of bexarotene, 60% developed cholesterol more than 300 mg/dL and 70% developed more than 2.5 times the normal level of triglycerides including 55% with triglycerides over 800 mg/dL.…”

Mycosis fungoides and sezary syndrome: An update

“…Bouwhuis et al [60] noted that their six reported patients had advanced disease that was at high risk for progression, and they did not infer causation. The discordance between skin improvement and development of nodal or visceral disease in these patients raised the question of whether bexarotene has effects on tissue adhesion markers, chemokines, and chemokine receptors resulting in redistribution or "compartmentalization" of the malignant T cells.…”

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

“…Bexarotene, a retinoid X-receptor (RXR)-selective retinoid, has recently been approved by the Food and Drug Administration (FDA) for cutaneous T-cell lymphoma including refractory MF and Sézary syndrome (4). There are only anecdotal reports on the treatment of CD30+ ALCL with bexarotene (5,6).…”