Department of Hematology, First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, Jiangsu 210029, China.
Funds:
This work was supported by Foundation of National Natural Science Foundation of China (81241074,81071946 and 81302040), Natural Science Foundation of Jiangsu Province (BK2012485), Jiangsu Province's
Medical Elite Program (RC201148)
a Project Funded by the Priority Academic Program Development of Jiangsu Higher Education Institutions, the Program for Development of Innovative Research Team in the First
Affiliated Hospital of NJMU, Clinical Research Program from Health Ministry of China (Key project 2010 to 2012), and Scientific Research Program for Public Interests from the Health Ministry of China (No.
201202017)
This study sought to analyze the clinical features and prognosis of multiple myeloma with isolated extramedullary relapse and with the absence of systemic progression. The clinical features and outcome were retrospectively analyzed in six multiple myeloma patients. These patients had secretory multiple myeloma at diagnosis. When relapsed, the dissociation between medullary and extramedullary response was detected. The serum or urine monoclonal component was extremely low or absent. The plasma cells in bone marrow were < 5%. All patients received new targeted therapies (thalidomide or bortezomib) before extramedullary relapse. It is difficult to achieve second remission for them. Even in those showing response, the duration of response was extremely short. The median of overall survival from diagnosis and from extramedullary relapse was 19 months and 6 months, respectively. The overall survival was significantly shorter compared to the patients without extramedullary involvement (84 months, P = 0.001). These patients exhibited a special and rare relapse pattern. Patients with this relapse pattern were resistant to current therapies, including novel targeted agents and associated with poor prognosis.