The TEMPI syndrome-Reference-Cited by-同舟云学术

The TEMPI syndrome

Published:2020-04-09 Issue:15 Volume:135 Page:1199-1203
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Sykes David B.¹^ORCID,O’Connell Casey²,Schroyens Wilfried³

Affiliation:

1. Massachusetts General Hospital, Boston, MA;

2. Keck School of Medicine, University of Southern California, Los Angeles, CA; and

3. Department of Hematology, Antwerp University Hospital, Antwerp, Belgium

Abstract

Abstract The TEMPI syndrome is a rare and acquired disorder characterized by 5 salient features, which compose its name: (1) telangiectasias; (2) elevated erythropoietin and erythrocytosis; (3) monoclonal gammopathy; (4) perinephric fluid collections; and (5) intrapulmonary shunting. Complete resolution of symptoms following treatment with plasma cell-directed therapy supports the hypothesis that the monoclonal antibody is causal and pathogenic. Understanding the basis of the TEMPI syndrome will depend on the identification of additional patients and a coordinated international effort.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/135/15/1199/1723202/bloodbld2019004216c.pdf

Reference33 articles.

1. Case records of the Massachusetts General Hospital. Case 23-2010. A 49-year-old man with erythrocytosis, perinephric fluid collections, and renal failure;Bazari;N Engl J Med,2010

2. The TEMPI syndrome--a novel multisystem disease;Sykes;N Engl J Med,2011

3. Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist;Rosado;Mod Pathol,2015

4. Monoclonal gammopathy of cutaneous significance: review of a relevant concept;Lipsker;J Eur Acad Dermatol Venereol,2017

5. Monoclonal gammopathy of clinical significance: a novel concept with therapeutic implications;Fermand;Blood,2018

Cited by 34 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. TEMPI syndrome: difficult to diagnose, “easy” to treat?;Annals of Hematology;2024-07-30

2. Плазмоклеточные опухоли в гематологических классификациях 2022 г.: WHO-HAEM5 (ВОЗ, 5-й пересмотр) и ICC (Международная консенсусная классификация). Взгляд клинициста;Clinical Oncohematology;2024-04-01

3. A rare case of TEMPI syndrome (telangiectasias, erythrocytosis, monoclonal gammopathy and ascites) associated with IgM monoclonal gammopathy;Leukemia & Lymphoma;2024-02-28

4. Complete Remission in a TEMPI Syndrome Treated with a Daratumumab, Lenalidomide, and Dexamethasone-Based Regimen: A Case Report;Case Reports in Oncology;2024-01-31

5. Recommandations 2024 de l’Intergroupe francophone du myélome sur la prise en charge des gammapathies monoclonales de signification indéterminée;Hématologie;2024-01-01