TEMPI syndrome: difficult to diagnose, "easy" to treat?

Autor: Fotiou D; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Solia E; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Theodorakakou F; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Nikolaou P; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Gakiopoulou C; 1st Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece., Psimenou E; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Papanikolaou A; Department of Hemopathology, Evangelismos Hospital, Athens, Greece., Dimopoulos MA; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece., Kastritis E; Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vas Sofias 80, Athens, 11528, Greece. ekastritis@gmail.com.; Laboratory of Pharmacology, School of Pharmacy, National and Kapodistrian University of Athens, Athens, 15771, Greece. ekastritis@gmail.com.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2024 Sep; Vol. 103 (9), pp. 3787-3793. Date of Electronic Publication: 2024 Jul 30.
DOI: 10.1007/s00277-024-05893-8
Abstrakt: 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.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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