TEMPI syndrome: difficult to diagnose, "easy" to treat?
Ann Hematol
; 103(9): 3787-3793, 2024 Sep.
Article
in En
| MEDLINE
| ID: mdl-39078435
ABSTRACT
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.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Polycythemia
Limits:
Aged
/
Humans
/
Male
Language:
En
Journal:
Ann Hematol
Journal subject:
HEMATOLOGIA
Year:
2024
Type:
Article
Affiliation country:
Greece