Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation

eJHaem

article

Davide Nappi¹  Martina Tauber²  David Sykes³ 

[1] Hematology and Bone Marrow Transplantation, Bolzano Hospital;Department of Pathology, Bolzano Hospital;Massachusetts General Hospital, Center for Regenerative Medicine

关键词: angiogenesis;    erythrocytosis;    inflammation;    MGUS;    multiple myeloma;   

DOI  :  10.1002/jha2.561

来源: Wiley

【 摘 要 】

The TEMPI syndrome is a very rare paraneoplastic syndrome associated with plasma cell dyscrasia and monoclonal gammopathy. First described in 2011, the pathophysiology of TEMPI syndrome is still unknown. Essential for diagnosis is to recognize the five clinical findings: telangiectasias, erythrocytosis and elevated serum erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting. Here we report a case of a woman with the coexistence of TEMPI and leukocytoclastic vasculitis, shedding light on a possible common inflammatory pathway involved in the pathogenesis of the syndrome.

【 授权许可】

Unknown