Cutaneous Castleman disease

Abstract

A 59-year-old Korean man was diagnosed in 1999 with multicentric plasma cell variant of Castleman disease (CD). He received CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy in 2000 with partial response followed by thalidomide, and then rituximab treatment without success, and finally high dose steroids. He presented to our clinic in 2007 for a second opinion regarding biopsy-proven refractory cutaneous involvement by CD. He had no B symptoms at that point and his main concerns were related to his skin lesions. His baseline positron emission tomography (PET) scan showed increased uptake by multiple lymph nodes. The constitutional symptoms of CD result from secretion of interleukin-6 (IL-6) by the hyperplastic lymph nodes. As CD is an IL-6 driven condition, we used siltuximab, a chimeric human-murine anti-IL-6 antibody for this orphan disease. Following written informed consent, the patient was enrolled in a Phase I study and was treated with the investigational agent siltuximab (CNTO328), 12 mg/kg intravenously every 2 weeks. Prior to this treatment the patient had scattered hyperpigmented CD lesions (top left); partial response had occurred by 6 months (top right) and after 24 months of siltuximab he had achieved an almost complete response (bottom), which has been maintained for more than 4 years of treatment. He has had no side effects and continues to receive silutuximab. His latest PET scan showed no abnormal uptake.