Relapsed multiple myeloma as TEMPI syndrome with good response to salvage lenalidomide and dexamethasone
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A new disease entity, TEMPI syndrome, characterized by the combination of telangiectasia, erythrocytosis and elevated serum erythropoietin level, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting, is included in the 2016 revised World Health Organization classification of tumors of hematopoietic and lymphoid tissues . It is hypothesized to be a result of abnormal plasma cell clones; however, its exact mechanism is unknown. Patients with TEMPI syndrome frequently suffer from distressing symptoms, and management is difficult since the standard treatment is yet to be elucidated. Herein, we report a case of a patient with TEMPI syndrome, who presented with severe fluid accumulation bilaterally in the perinephric space and polycythemia at the time of IgA-kappa multiple myeloma relapsed, and responded dramatically to lenalidomide and dexamethasone.
All authors would like to acknowledge the Division of Hematology and Oncology, Department of Internal Medicine, and China Medical University Hospital for its support while providing a valuable contribution to the work.
SHL carried out the data gathering and drafted the manuscript. SPY provided the concept and data analysis. All authors read and approved the final manuscript.
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Conflict of interest
The authors declare that they have no conflict of interest.
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