TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis

Xu, Jian; Liu, Wenqi; Fan, Fengjuan; Zhang, Bo; Zhao, Fei; Hu, Yao; Sun, Chunyan

doi:10.3389/fendo.2022.886961

Cited by 8 publications

(5 citation statements)

References 39 publications

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“…For instance, the typical manifestations of TEMPI syndrome include the pentad of telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. However, not all features are commonly present at the onset of the disease [ 91 ]. Moreover, other important features not included in the TEMPI acronym, such as venous thrombosis, spontaneous intracranial hemorrhage, ascites, and other cutaneous [ 99 ] or ocular signs [ 101 ], may also appear.…”

Section: Discussion and Practical Applicationsmentioning

confidence: 99%

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Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Ríos-Tamayo

Lahuerta

Martínez‐López

et al. 2022

Cancers

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Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.

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Section: Discussion and Practical Applicationsmentioning

confidence: 99%

“…The first patient with TEMPI syndrome was described in 2010 [ 87 ]. It is an ultrarare novel multisystem disease [ 88 , 89 , 90 , 91 ]. Only 22 patients were reported at the end of 2019.…”

Section: Mgcs As a Global And Unifying Conceptmentioning

confidence: 99%

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Ríos-Tamayo

Lahuerta

Martínez‐López

et al. 2022

Cancers

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“…It is characterized by a plethora of symptoms made up with initials form the acronym Telangiectasias, elevated erythropoietin (EPO) level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting (TEMPI). Ever since that date, < 30 cases have been reported 2 whose study helps get a hold of the etiopathogenic mechanisms involved, which highlights the importance of the monoclonal component as the triggering factor of this clinical course 3,4 . Response to drugs that reduce the clone of plasma cells responsible for monoclonal paraprotein like daratumumab, the proteasome inhibitor bortezomib, and lenalidomide support this hypothesis.…”

Section: Introductionmentioning

confidence: 99%

The TEMPI syndrome: evolution and response to bortezomib and daratumumab treatment

Núñez¹,

López²,

Galán³

et al. 2023

SANGREE

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TEMPI syndrome is considered a plasma cell neoplasm with associated paraneoplastic syndrome that generates a set of symptoms that define it: telangiectasias, erythrocytosis with elevates erythropoietin, monoclonal gammopathy, accumulation of perinephric fluid, and intrapulmonary shunting. Its etiopathogenesis seems to be due to the effect of paraprotein. It responds quite well to bortezomib an daratumumab, drugs used in the case that we present. It is a rare process that is often not taken into account, which delays the diagnosis, so it is important to communicate cases so that their knowledge can be considered in the differential diagnosis of some of the symptoms that define it.

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“…TEMPI (telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) syndrome is a rare multisystemic disease classified as a monoclonal gammopathy of cutaneous significance. 1,2 The diagnosis of TEMPI syndrome is a challenging one, as patients oftentimes have a complex clinical presentation that can be misdiagnosed as other conditions associated with erythrocytosis, such as polycythemia vera (PV), which results in delayed treatment and clinical deterioration. 3 Treatment with plasma-cell-directed therapies, including bortezomib-based regimens, daratumumab, lenalidomide, and autologous stem cell transplantation, have been shown to result in a dramatic resolution of symptoms.…”

Section: Introductionmentioning

confidence: 99%

TEMPI syndrome: A clinical, light‐microscopic and phenotypic evaluation with review of the literature

Kalomeris,

Grossman,

Tepler

et al. 2023

J Cutan Pathol

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Background and objectivesTEMPI (telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonaryshunting) syndrome is a rare multisystemic disease classified as a monoclonal gammopathy of cutaneous significance. The pathogenesis and etiology of TEMPIare not well known because of the rarity of this disorder. Although telangiectasias are the hallmark of this syndrome, skin biopsies are rarely performed. We aim to further characterize TEMPI syndrome through the evaluationof a skin biopsy.MethodsWe reviewed the histopathology and immunophenotypic profile of a skin biopsy from a 53‐year‐oldwoman diagnosed with TEMPI syndrome. Other components of her syndromic complex included an IgA myeloma, elevated vascular endothelial growth factor (VEGF), and erythrocytosis.ResultsA biopsy showed prominent vascular ectasia with some degree of microvascular basement membranezone thickening. Our patient had a reduction in neoplastic plasma cell burdenand clearing of her telangiectasias following myeloma directed treatment.ConclusionsTEMPI can beviewed as a reactive vascular paraneoplastic syndrome in the setting of a plasma cell dyscrasia. Elaboration of VEGF from neoplastic plasma cells is likely pathogenetically implicated and appears to be a common link that explains other vascular lesions associated with monoclonal gammopathy syndromes.

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TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis

Cited by 8 publications

References 39 publications

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

The TEMPI syndrome: evolution and response to bortezomib and daratumumab treatment

TEMPI syndrome: A clinical, light‐microscopic and phenotypic evaluation with review of the literature

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