Extracorporeal photopheresis for the treatment of erythrodermic cutaneous T‐cell lymphoma: a single center clinical experience with long‐term follow‐up data and a brief overview of the literature

Abstract

Extracorporeal photopheresis (ECP) is a therapeutic procedure in which leukapheresed peripheral blood mononuclear cells are exposed to ultraviolet A in the presence of the photosensitizer 8-methoxypsoralen and then reinfused. Several guidelines recommend ECP as a treatment of choice in erythrodermic primary cutaneous T-cell lymphomas (E-CTCL). However, the level of evidence is low due to the rarity of this disease and the lack of randomized controlled trials. We performed a review of the English literature, restricting our analysis to studies including erythrodermic patients and more than 10 cases. Based on these criteria, we identified 28 studies, with a total of 407 patients. The median response rate in erythrodermic patients was 63% (range 31-86%), with a complete response rate ranging between 0 and 62% (median 20%). In our experience, we treated 51 patients with E-CTCL since 1992. A clinical response was obtained in 32 of 51 patients (63%), with a 16% complete response rate. The median time for response induction was eight months (range: 1-23). The median response duration was 22.4 months (range six months to 11 years). The treatment was generally well tolerated without systemic toxicities grade III-IV. The pretreatment parameters significantly associated with a higher likelihood to obtain a clinical response were the B-score in the peripheral blood, CD4/CD8 ratio, and amount of circulating CD3+CD8+ cells. Literature data together with our personal experience clearly support the clinical activity and tolerability of ECP in patients with E-CTCL. Prospective controlled clinical trials are strongly recommended to better document the evidence.

Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sézary Syndrome

“…Since then, the responses of more than 518 ECP-treated patients with erythrodermic MF/SS have been published and summarized with a wide range of response rates from 33% to 74%. 44,57 Many of these patients were treated with photopheresis in combination with other therapies. In addition, none of the patients were part of prospective randomized controlled clinical trials.…”

“…57,68 They include relatively low tumor load of malignant T cells in the blood, lymph nodes, and skin; peripheral blood involvement; relatively intact immune system; erythroderma; and plaques covering less than 10% to 15% of the total skin surface 68 (Table 1).…”

“…In a recent series of 51 patients with erythrodermic MF/SS treated with ECP, the following adverse effects were reported using the National Cancer Institute Common Toxicity Criteria system: transient grade I hypotension (12%), grade I-II anemia (6%), hypokalemia (4%), and 1 urticarial eruption interpreted as a drug reaction to either 8-MOP or heparin. 57 Discomfort and mild hematomas at venipuncture sites are not uncommon and can be relieved with pressure bandages and ice compresses. 17 To avoid catheter-related infections, intermittent peripheral venous access is preferred over indwelling catheters because of the high rate of Staphylococcus aureus colonization on the skin in patients with erythrodermic MF/SS.…”

Extracorporeal Photopheresis—An Overview

“…In SS atypical mononuclear cells with a cerebriform nucleus (Sézary cells) appear which can be found in the skin, peripheral blood and lymph nodes. SS usually has a bad prognosis with a 5-year survival rate of 24% (27, 28). Initial treatment of CTCL is directed at the cutaneous involvement to improve quality of life and minimize the risk of reoccurrence.…”

Extracorporeal Photopheresis: Scientific and Technical Considerations for Improving Clinical Management of Patients

“…In these disseminated erythrodermic CTCL, the median response reported is 63% (range 30-80%) [33]. The schedule of treatment is now well-standardized with an intensive induction phase consisting of two consecutive days every two weeks followed by observation of a response in maintenance/ sustaining phase.…”