Extracorporeal photopheresis in Sézary syndrome: hematologic parameters as predictors of response

Abstract

Data were analyzed from 23 patients with Sé zary syndrome (defined by erythroderma, more than 10% circulating atypical mononuclear cells, and peripheral blood T-cell clone) undergoing monthly extracorporeal photopheresis as the sole therapy for up to 1 year. The cohort showed a significant reduction of skin scores during treatment (P ‫؍‬ .001). Thirteen patients (57%) achieved a reduction in skin score greater than 25% from baseline at 3, 6, 9, or 12 months (responders). Reduction in skin score correlated with reduction in the Sé zary cell count as a percentage of total white cell count (P ‫؍‬ .03). Responders and nonresponders were compared. None of the measured parameters was significantly different between the 2 groups. It was assessed whether any of the baseline parameters predicted outcome. A higher baseline lymphocyte count was significantly associated with a decrease in skin score at 6 months (P < .05). A higher baseline Séz-ary cell count as a percentage of total white cell count predicted a subject was more likely to be a responder after 6 months of treatment (P ‫؍‬ .021). No other parameters predicted responder status. These data show that the modest falls in CD4, CD8, and Sé zary cell counts were seen in all patients and might have resulted from lymphocyte apoptosis. This mechanism could explain the more favorable response seen in patients with higher percentages of Sé zary cells in the peripheral blood. Alternatively, minimum tumor burden might be required for the induction of a cytotoxic response. Analysis of tumor-specific cytotoxic T cells is needed to investigate these possibilities further. (Blood. 2001;98:1298-1301)

EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome

“…134 The authors noted that responders were more likely to have a higher baseline lymphocyte count and a higher proportion of Sé zary cells in the total leukocyte count than non-responders, whereas others have reported optimal responses in patients with a lower CD4/CD8 ratio. 134,135 The use of ECP to treat MF/SS, including ECP combination modalities, has been recently reviewed by Knobler and Jantschitsch. 77 Other recommended treatment options include immunotherapy, BRMs, and chlorambucil plus prednisone.…”

Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sézary syndrome

“…16,17 Extracorporeal photopheresis may also be used successfully but is not generally available. 18,19 Once the disease becomes refractory to topical therapy, interferon-␣, bexarotene, single-agent chemotherapy, or combination chemotherapy may be given, but the duration of response is often less than 1 year, and ultimately all patients have relapses and the disease becomes refractory. 8,[20][21][22][23][24] Response rates following combined modality therapy with TSEB and chemotherapy/ interferon-␣ appear similar to those of other therapies.…”

Extracorporeal photochemotherapy for the treatment of steroid-resistant chronic GVHD

“…CTCL responds to biological response modification, 5 and ECP produces a high clinical response rate. [6][7][8] It is felt that this therapy not only augments the function of monocytes but also induces the malignant T cells to undergo a high rate of apoptosis, exerting an antitumor effect through cytokine modulation and modification. 9,10 In chronic GVHD, ECP has been tested quite extensively in small cohorts of patients, and responses were observed in skin, liver, gastrointestinal (GI) tract, mouth, eye, and lung.…”