%0 Case Reports
%T TEMPI syndrome: difficult to diagnose, "easy" to treat?
%A Fotiou D
%A Solia E
%A Theodorakakou F
%A Nikolaou P
%A Gakiopoulou C
%A Psimenou E
%A Papanikolaou A
%A Dimopoulos MA
%A Kastritis E
%J Ann Hematol
%V 103
%N 9
%D 2024 Sep 30
%M 39078435
%F 4.03
%R 10.1007/s00277-024-05893-8
%X TEMPI syndrome is a rare, acquired disorder with multisystemic manifestations. It is classified as a plasma cell disorder and is characterized by telangiectasias, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunt. Even though TEMPI's pathophysiology remains elusive, it responds to anti-myeloma therapy indicating that the monoclonal protein or clone plays a key role. We present a challenging case of a 73-year-old man with erythrocytosis and deteriorating renal function with nephrotic-range proteinuria in whom after extensive work up, the diagnosis of TEMPI syndrome was made. He was received treatment with daratumumab-bortezomib-cyclophosphamide and dexamethasone (Dara-VCD) and achieved a hematological and clinical response. We also report preliminary data on a multiplex assay for cytokines and growth factors for two patients with TEMPI syndrome and note lower levels for non-specific innate immunity related cytokines. A direct link between renal impairment and TEMPI syndrome is not currently established; cytokine deregulation could potentially be involved in the ischemic changes observed in the renal biopsy of our patient.