Treatment options for cutaneous T-cell lymphoma

Because of the rarity of cutaneous T-cell lymphoma, no standard treatments have been established. Management and treatment of patients with cutaneous T-cell lymphoma should be individualized and, in some cases, watchful waiting may be appropriate. Treatment may only be necessary if hemophagocytic syndrome develops.[58]

There is no standard treatment regimen for SPTCL. Spontaneous remissions have been observed, particularly in younger children. Older children, however, may have a course of disease that is complicated by hemophagocytic syndrome. First-line treatment consists of either chemotherapy or immunomodulatory drugs. Chemotherapy was the mainstay of treatment before 2019. Immunomodulatory therapy or observational follow-up became the mainstay of treatment after this time. Immunomodulatory agents include steroids combined with cyclosporine A or ruxolitinib. In a series of 18 patients, the CR rate was 71.4% for patients treated with these immunomodulatory agents.[55][Level of evidence C3]

An oral retinoid (bexarotene) has been reported to be active against SPTCL in a series of 15 patients from three institutions.[59] In a series of 11 pediatric patients, aggressive polychemotherapy was used in all patients. Nine of 11 patients sustained clinical remission, with a median follow-up of 3.5 years.[56] Additional treatment options include high-dose steroids, bexarotene, denileukin diftitox, multiagent chemotherapy, and HSCT.[53,58-63]

Primary cutaneous anaplastic large cell lymphoma usually does not express ALK and may be treated successfully with surgical resection and/or local radiation therapy without systemic chemotherapy.[64] There are reports of surgery alone also being curative for patients with ALK-positive cutaneous anaplastic large cell lymphoma, but extensive staging and vigilant follow-up is required.[65,66]