What is the role of extracorporeal photopheresis (ECP) in the treatment of cutaneous T-cell lymphoma (CTCL)?

Updated: Nov 21, 2016
  • Author: Vikas Shrivastava, MD; Chief Editor: William D James, MD  Dirk M Elston, MD  more...
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Answer

Answer

CTCL is a group of lymphoproliferative disorders caused by clonally derived, skin-homing, malignant T cells. The most common of these disorders is mycosis fungoides (60%) and its leukemic variant, Sézary syndrome (5%), which is an aggressive variant that has peripheral blood, lymph node, and sometimes internal organ involvement. [8] Only 5 therapies have been approved by the FDA for the treatment of mycosis fungoides and Sézary syndrome, of which extracorporeal photopheresis (ECP) was the earliest. [8]

As noted by Knoebler et al, a number of studies have been published that support the use of ECP in mycosis fungoides and Sézary syndrome both as monotherapy and in combination with other treatment modalities such as interferons, granulocyte macrophage colony-stimulating factor (GM-CSF), and systemic retinoids. [1] In addition to the improvement in cutaneous manifestations of CTCL, ECP has been demonstrated to cause a decrease in the burden of peripheral blood disease and lymphadenopathy. [12, 13, 14] While survival benefit has not been prospectively established, data do indicate improvement in survival. [8]

A clinical profile of patients who are more likely to respond to ECP has been developed and was summarized by Knoebler et al. [1] The presence of a discrete population of circulating Sézary cells, short duration of disease (< 2 y), preserved number of CD8+ cytotoxic lymphocytes (CD4:CD8 ratio < 10), [7] absence of lymphadenopathy and internal disease, WBC count less than 20,000/µL, absence of prior chemotherapy, and limited number of skin plaques make response more likely. Normal pretreatment lactate dehydrogenase has also shown higher response rate. [7] Lastly, as shown by Zic, the strongest predictor of long-term treatment response is 50% or more reduction in skin lesions by 6 months. [1, 8]

ECP has classically been used in later stage mycosis fungoides and Sézary syndrome. However, because of the efficacy, tolerability, and safety of this treatment modality, strong consideration is being given to use earlier in disease and in other subtypes. [8] In fact, current National Comprehensive Cancer Network (NCCN) guidelines include ECP as a first-line treatment for early, refractory disease. [1]


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