Journal Logo


Photopheresis in the treatment of the erythrodermic CTCL patient

Knobler, R.

Author Information
doi: 10.1097/01.cmr.0000382786.82300.50
  • Free

Extracorporeal photopheresis (ECP) is an apheresis based therapy which was originally conceived by Edelson et al. in 1983 for the systemic treatment of cutaneous T-cell lymphoma (CTCL), the Sezary syndrome variant. Since its original concept until 2010 it has been found to be of significant clinical value in this primary indication as well as additional T-cell mediated diseases. Soon after its introduction it was approved in 1985 by the FDA (USA) for the palliative treatment of CTCL. In 2006 it was included in the EORTC CLTF treatment guidelines to be one of the first line therapies for the treatment of the Sezary syndrome (erythrodermic) variant of CTCL.

Evidence collected in the past 20 years in over 1000 CTCL patients has demonstrated that this treatment modality can have a very significant effect on the clinical course of a subset of CTCL patients, particularly the erythrodermic, Sezary Syndrome variant. Newest clinical studies and data not only support the published evidence for its efficacy as a monotherapy but also as a therapy that can be combined with multiple biological response modifiers including interferon alfa, GMCSF, IL12, Interferon Gamma, as well as total skin electron therapy (TSEB) and systemic chemotherapy. The evidence available, though positive, remains controversial within the medical community. A number of very well-designed multi-center trials which have been lacking since the first publication by Edelson et al. are presently being planned so that hopefully a number of open questions will be resolved with greater clarity in the near future. The definition of the exact entry treatment criteria which promise optimal response with optimal treatment frequency remain equally to be conclusively defined. Early intervention within 2 years of the appearance of first symptoms, low tumor load, absence of high count leukemia and absence of further transformation are criteria that help predict patients most likely to respond but do not exclude necessarily patients that have more advanced disease and who may be candidates for combination therapy.

This innovative approach for the treatment of CTCL has certainly opened new avenues of therapy and thought in photoimmunology and photomedicine. Clearly the very low side effect profile of this therapy has made it more attractive than the chemotherapeutic and immunosuppressive substances that are presently available or in experimental protocols. If and when the mechanisms of action are further better understood and appropriate studies investigating different treatment schedules and different combination therapies and modifications of its present form are performed, the place of photopheresis in the therapeutics of CTCL, particularly the erythrodermic Sezary syndrome variant, as well as other T-cell mediated diseases and oncology will be better defined.

© 2010 Lippincott Williams & Wilkins, Inc.