Mycosis fungoides (MF) and Sézary syndrome (SS), are clonal T-cell lymphomas arising from the skin. When someone has MF, malignant cells in the blood travel to the skin, causing lesions that appear as an itchy rash. That rash can ultimately transform into tumors and malignant cells can spread to other organs in the body. Extra-skin involvement may occur in lymph nodes, liver, spleen, lungs and blood (Sézary cells). SS is the leukemic presentation of this disease in which patients have significant circulating Sézary cells in the blood and diffuse erythroderma.  Patients often suffer from life-altering pruritus and frequent skin infections.

The management of patients with MF and SS is complex and unique to each patient. Skin-directed therapy and mild systemic biological therapy is commonly used for early stage disease. However, stronger biological therapy, antibodies or its conjugates, systemic chemotherapies and radiation are usually required for progressive or recurrent disease. For patients with advanced stage disease, the available treatments only work for 4-6 months and the cancer cells become more aggressive after each treatment. Patients with stage IV disease generally have <2 years median survival. "With these patients, you definitely need to think about a permanent change—specifically, a stem cell transplant to replace the immune system with donor cells which would keep the disease permanently in check." said Dr. Youn Kim, director of Stanford’s Multidisciplinary Cutaneous Lymphoma Clinic. To find a long term solution for these patients, Drs. Wen-Kai Weng and Sally Arai teamed up with members of the Stanford Cutaneous Lymphoma Clinic to conduct a clinical trial using a novel allogeneic transplant protocol. In this study, total skin electron beam therapy (TSEBT) along with total lymphoid irradiation (TLI) and anti-thymocyte globulin (ATG) is used as conditioning regimen. Patients receive 12-36 Gy of TSEBT depending on the previous exposure for tumor reduction in the skin. While the TSEBT is a critical part of this regimen, disease control in the blood and lymph node compartments prior to transplant has been challenging. To achieve the best pre-transplant disease control, the entire team works together to manage these pre-transplant patients. Each week, Drs. Kim, Weng, Richard Hoppe (Radiation Oncology), Lynn Million (Radiation Oncology) and Sunil Reddy (Medical Oncology) hold a clinic and see patients together. “Our collaborative approach extends beyond a typical tumor board” says Weng, “Our team doesn’t just discuss patients, we treat them together to get them ready for transplant.”

Thus far, 29 patients have received allogeneic HCT under this protocol. The median age was 62 y/o (range 20-74). These patients have been heavily treated with a median of 5 prior systemic treatments. All patients had active disease at the time of conditioning regimen. Patients tolerated this regimen very well with one-year transplant-related mortality of 3%. The estimated 2-year EFS was 52% and 2-year OS was 74%. In addition, HTS of T-cell receptor has shown that 50% of the patients have achieved molecular remission in both blood and skin post-transplant. 

Based on these encouraging results, we believe this teamwork approach has provided a patient-specific, multi-modality management plan for these challenging patients.