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P a g e s f o r l o g g e d o u t e d i t o r s l e a r n m o r e ● C o n t r i b u t i o n s ● T a l k
( T o p )
1 S i g n s a n d s y m p t o m s
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A p p e a r a n c e
F r o m W i k i p e d i a , t h e f r e e e n c y c l o p e d i a
( R e d i r e c t e d f r o m T e m p i s y n d r o m e )
TEMPI syndrome Other names Telangiectasia-erythrocytosis-monoclonal gammopathy-perinephric-fluid collections-intrapulmonary shunting syndrome
TEMPI syndrome is an orphan disease where the patients share five characteristics from which the acronym is derived: telangiectasias , elevated erythropoietin and erythrocytosis , monoclonal gammopathy , perinephric fluid collection, and intrapulmonary shunting .
Signs and symptoms
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TEMPI
Symptom
T Telangiectasias
E Elevated Erythropoietin and Erythrocytosis
M Monoclonal gammopathy
P Perinephric fluid collections
I Intrapulmonary shunting
The patients were all diagnosed at middle age. A monoclonal gammopathy was implicated in all patients tested.[1]
Cause
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The cause of the syndrome is unknown. The abnormal plasma-cell clone and/or the monoclonal gammopathy are suggested to be triggers of the disease.[2]
Diagnosis
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The diagnosis is based on the five characteristics described above.[2]
Treatment
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Complete and partial disappearance of the symptoms of the TEMPI syndrome was reported with the drugs bortezomib ,[3] daratumumab [4] [5]
History
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In 2010, the case of a man with unexplained erythrocytosis and perinephric fluid collection as main features was described in the Case Records of the Massachusetts General Hospital .[6]
References
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^ a b Sykes, David B.; O'Connell, Casey; Schroyens, Wilfried (2020-04-09). "The TEMPI syndrome" . Blood . 135 (15 ): 1199–1203. doi :10.1182/blood.2019004216 ISSN 1528-0020 . PMID 32108223 .
^ Schroyens, Wilfried; O'Connell, Casey; Sykes, David B. (2012). "Complete and Partial Responses of the TEMPI Syndrome to Bortezomib" (PDF) . N Engl J Med 367 (8 ): 778–780. doi :10.1056/NEJMc1205806 . PMID 22913703 .
^ Sykes, David B.; Schroyens, W. (2018). "Complete Responses in the TEMPI Syndrome after Treatment with Daratumumab". N Engl J Med 378 (23 ): 2240–2242. doi :10.1056/NEJMc1804415 . PMID 29874534 . S2CID 205064988 .
^ Kenderian, S.S..; Rosado, F.G; Sykes, D.B.; Hoyer, J.D.; Lacy, M.Q. (2015). "Long-term complete clinical and hematological responses of the TEMPI syndrome after autologous stem cell transplantation" . Leukemia 29 12 ): 2414–2416. doi :10.1038/leu.2015.298 PMID 26500143 .
^ Bazari, Hasan; Attar, Eyal C.; Dahl, Douglas M.; Uppot, Raul N.; Colvin, Robert B. (2010). "Case Records of the Massachusetts General Hospital. Case 23-2010: A 49-Year-Old Man with Erythrocytosis, Perinephric Fluid Collections, and Renal Failure". N Engl J Med 363 (5 ): 463–475. doi :10.1056/NEJMcpc1004086 . PMID 20818867 .
External links
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Classification
External resources
R e t r i e v e d f r o m " https://en.wikipedia.org/w/index.php?title=TEMPI_syndrome&oldid=1218325448 " C a t e g o r i e s : ● R a r e s y n d r o m e s ● S y n d r o m e s o f u n k n o w n c a u s e s ● S y n d r o m e s a f f e c t i n g b l o o d ● S y n d r o m e s a f f e c t i n g t h e v a s c u l a r s y s t e m H i d d e n c a t e g o r i e s : ● A r t i c l e s w i t h s h o r t d e s c r i p t i o n ● S h o r t d e s c r i p t i o n i s d i f f e r e n t f r o m W i k i d a t a
● T h i s p a g e w a s l a s t e d i t e d o n 1 1 A p r i l 2 0 2 4 , a t 0 1 : 3 5 ( U T C ) . ● T e x t i s a v a i l a b l e u n d e r t h e C r e a t i v e C o m m o n s A t t r i b u t i o n - S h a r e A l i k e L i c e n s e 4 . 0 ;
a d d i t i o n a l t e r m s m a y a p p l y . B y u s i n g t h i s s i t e , y o u a g r e e t o t h e T e r m s o f U s e a n d P r i v a c y P o l i c y . W i k i p e d i a ® i s a r e g i s t e r e d t r a d e m a r k o f t h e W i k i m e d i a F o u n d a t i o n , I n c . , a n o n - p r o f i t o r g a n i z a t i o n . ● P r i v a c y p o l i c y ● A b o u t W i k i p e d i a ● D i s c l a i m e r s ● C o n t a c t W i k i p e d i a ● C o d e o f C o n d u c t ● D e v e l o p e r s ● S t a t i s t i c s ● C o o k i e s t a t e m e n t ● M o b i l e v i e w
