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TEMPI syndrome: difficult to diagnose, "easy" to treat?
Fotiou, Despina; Solia, Eirini; Theodorakakou, Foteini; Nikolaou, Panagiota; Gakiopoulou, Charikleia; Psimenou, Erasmia; Papanikolaou, Asimina; Dimopoulos, Meletios A; Kastritis, Efstathios.
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  • 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.
Ann Hematol ; 103(9): 3787-3793, 2024 Sep.
Article en 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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Policitemia Límite: Aged / Humans / Male Idioma: En Revista: Ann Hematol Asunto de la revista: HEMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Grecia Pais de publicación: Alemania
Texto completo
Añadir a Mi BVS
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Policitemia Límite: Aged / Humans / Male Idioma: En Revista: Ann Hematol Asunto de la revista: HEMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Grecia Pais de publicación: Alemania