Yale Dermatology Photopheresis Unit

Michael Girardi, MD Director

Staff

• Francine Foss, MD - Associate Director
• Kacie Carlson, PA-C - Associate
• Inger Christensen, RN - Clinical Coordinator
• Maureen Alvarez, RN
• Laura Hinners, RN
• Niki Dobbs, RN
• Annette Myers - Administrative Assistant

The Photopheresis Center is under the direction of Michael Girardi, MD, Francine Foss, MD, Kacie Carlson, PA-C, and Inger Christensen, RN, the clinical coordinator. Maureen Alvarez, RN, Laura Hinners, RN, and Niki Dobbs, RN round out the staff of nurses who administer photopheresis to patients on a daily outpatient basis. The unit performs close to 1000 treatments per year.

Cutaneous T-cell lymphoma

When it was first described in the nineteenth century, cutaneous T-cell lymphoma looked like a fungus infection of the skin with large, mushroom-like protuberances, so it was called "mycosis fungoides." In the 1980s, research by Dr. Richard Edelson, Chairman of the Department of Dermatology at the Yale School of Medicine, proved that the disease was caused by a malignancy of the lymphocytes, and he renamed it cutaneous T-cell lymphoma or CTCL. CTCL is currently a central research interest of the Department of Dermatology and the focus of a well-organized inter-disciplinary group of scientists and clinicians.

  Photopheresis has become an important option for patients with advanced CTCL
  who do not respond to standard treatments.

This potentially dangerous form of lymphoma affects more than 10,000 people every year,which makes it the most common form of cancer of white blood cells of adults. At Yale, nearly 250 new CTCL patients are seen annually. When CTCL is untreated, the cancerous lymphocytes of CTCL can spread through the blood to other internal organs and distant skin sites. In the early stage, Yale treats CTCL successfully with every available modality including electron beam radiation and PUVA (psoralen and ultraviolet-A radiation). Other therapeutic options under study include new chemotherapy agents, interferon, and a new process called photopheresis. This procedure, invented by Dr. Edelson in the 1980s, is often called "extracorporeal photochemotherapy." Photopheresis has become an important option for patients with advanced CTCL who do not respond to standard treatments.

Photopheresis - Extracorporeal Photoimmunotherapy

The method of photopheresis is simple: the patient is connected to a machine that withdraws a quantity of blood in a manner similar to kidney dialysis. The machine, which is the size of a dishwasher, separates the blood into red blood cells, white blood cells, and plasma. The white blood cells and part of the plasma are mixed with a light activatable drug called 8-Methoxypsoralen (8-MOP). The blood and 8-MOP are irradiated with ultraviolet light within the machine before being combined with the other native blood components and returned to the patient.

8-Methoxypsoralen (8-MOP) is a photoactivatable drug that is used to treat a variety of skin diseases and lymphomas. To treat skin diseases, after oral ingestion of the drug the affected areas(s) of skin is exposed to the activating effects of long wavelength ultraviolet radiation (UVA, 320-400nm). The treated blood is returned to the patient. After treatments at monthly intervals, the best responders (25% of all subjects) begin to show a response after 3.5 months of therapy. Because only a small portion of the patient's malignant cells are ever treated it has been assumed that an immune response has been induced. Our department is investigating the impact of the formation of 8-MOP DNA photoadduct on transcription factor induction(such as NfkB) as well as the stimulation of cytokine secretion that may participate in the observed therapeutic responses. The studies will involve the correlation of in vitro and in vivo analyses.

When the new knowledge gained from current photopheresis research is fully translated into clinical practice, it is conceivable that people with many other immune illnesses may benefit. Preliminary tests in a range of studies from around the world suggest that photopheresis might be beneficial in "T-cell mediated autoimmune diseases" such as pemphigus, lupus erythematosus, multiple sclerosis, rheumatoid arthritis, scleroderma, and most recently, cardiac transplantation and graft versus host disease. In addition, the Oncology Section of the Yale Cancer Center is doing a pilot study to evaluate photopheresis in patients with sever solid tumors. Currently 900-1000 patients are treated yearly in the outpatient center.