TEMPI syndrome is a recently described entity characterized by Telangiectasias, Erythrocytosis with elevated erythropoietin, Monoclonal gammopathy, Perinephric fluid collections and Intrapulmonary shunting. The prevalence of the disease is not known and few cases have been reported in literature. Treatment for TEMPI syndrome has been varied. Mayo Clinic researchers, first author Martha Lacy, M.D., discuss a patient with TEMPI syndrome that had a dramatic and durable complete clinical and hematological response after high dose chemotherapy and autologous stem cell transplantation (ASCT). The research was published in the journal Leukemia.
A 49-year-old female was referred to Mayo Clinic for further evaluation of progressive hypoxia requiring the continuous use of high concentrations of oxygen. She had a 15-year history of erythrocytosis, thought to be related to Polycythemia Vera, and has been treated with intermittent phlebotomy since then.
Based on the hypothesis that TEMPI syndrome may be directly related to plasma cell dyscrasia and as ASCT has been associated with excellent long-term outcomes in other non-malignant plasma cell proliferative disorders, Mayo Clinic researchers we elected to offer ASCT to the patient.
Clinical improvement was noted starting few weeks post transplantation. After 100 days of ASCT, progress showed hematologic complete remission with negative serum protein electrophoresis and immunofixation, and normalization of the free light chain. Highly sensitive flow cytometry was negative for any plasma cell clonality. The patient had dramatic improvement in the TEMPI syndrome with resolution of the visible telangiectasias, disappearance of perinephric fluid collections, and normalization of erythrocyte count. Follow-up at one and two years post ASCT continued to show complete hematological and clinical response and she continues to be asymptomatic.
This case demonstrates that TEMPI syndrome is consistent with a paraneoplastic process related to plasma cell dyscrasia. This case also proves that ASCT is a viable option for these patients and results in complete clinical and hematological responses. It can also result in dramatic and long-term responses associated with disappearance of the monoclonal protein and all the paraneoplastic manifestations of the disease.