Inherited thrombocytopenias : an updated guide for clinicians
Copyright © 2020 Elsevier Ltd. All rights reserved..
The great advances in the knowledge of inherited thrombocytopenias (ITs) made since the turn of the century have significantly changed our view of these conditions. To date, ITs encompass 45 disorders with different degrees of complexity of the clinical picture and very wide variability in the prognosis. They include forms characterized by thrombocytopenia alone, forms that present with other congenital defects, and conditions that predispose to acquire additional diseases over the course of life. In this review, we recapitulate the clinical features of ITs with emphasis on the forms predisposing to additional diseases. We then discuss the key issues for a rational approach to the diagnosis of ITs in clinical practice. Finally, we aim to provide an updated and comprehensive guide to the treatment of ITs, including the management of hemostatic challenges, the treatment of severe forms, and the approach to the manifestations that add to thrombocytopenia.
Medienart: |
E-Artikel |
---|
Erscheinungsjahr: |
2021 |
---|---|
Erschienen: |
2021 |
Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
---|---|
Enthalten in: |
Blood reviews - 48(2021) vom: 15. Juli, Seite 100784 |
Sprache: |
Englisch |
---|
Beteiligte Personen: |
Pecci, Alessandro [VerfasserIn] |
---|
Links: |
---|
Themen: |
Bleeding disorders |
---|
Anmerkungen: |
Date Completed 03.09.2021 Date Revised 03.09.2021 published: Print-Electronic Citation Status MEDLINE |
---|
doi: |
10.1016/j.blre.2020.100784 |
---|
funding: |
|
---|---|
Förderinstitution / Projekttitel: |
|
PPN (Katalog-ID): |
NLM318830817 |
---|
LEADER | 01000naa a22002652 4500 | ||
---|---|---|---|
001 | NLM318830817 | ||
003 | DE-627 | ||
005 | 20231225170159.0 | ||
007 | cr uuu---uuuuu | ||
008 | 231225s2021 xx |||||o 00| ||eng c | ||
024 | 7 | |a 10.1016/j.blre.2020.100784 |2 doi | |
028 | 5 | 2 | |a pubmed24n1062.xml |
035 | |a (DE-627)NLM318830817 | ||
035 | |a (NLM)33317862 | ||
035 | |a (PII)S0268-960X(20)30134-X | ||
040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
041 | |a eng | ||
100 | 1 | |a Pecci, Alessandro |e verfasserin |4 aut | |
245 | 1 | 0 | |a Inherited thrombocytopenias |b an updated guide for clinicians |
264 | 1 | |c 2021 | |
336 | |a Text |b txt |2 rdacontent | ||
337 | |a ƒaComputermedien |b c |2 rdamedia | ||
338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
500 | |a Date Completed 03.09.2021 | ||
500 | |a Date Revised 03.09.2021 | ||
500 | |a published: Print-Electronic | ||
500 | |a Citation Status MEDLINE | ||
520 | |a Copyright © 2020 Elsevier Ltd. All rights reserved. | ||
520 | |a The great advances in the knowledge of inherited thrombocytopenias (ITs) made since the turn of the century have significantly changed our view of these conditions. To date, ITs encompass 45 disorders with different degrees of complexity of the clinical picture and very wide variability in the prognosis. They include forms characterized by thrombocytopenia alone, forms that present with other congenital defects, and conditions that predispose to acquire additional diseases over the course of life. In this review, we recapitulate the clinical features of ITs with emphasis on the forms predisposing to additional diseases. We then discuss the key issues for a rational approach to the diagnosis of ITs in clinical practice. Finally, we aim to provide an updated and comprehensive guide to the treatment of ITs, including the management of hemostatic challenges, the treatment of severe forms, and the approach to the manifestations that add to thrombocytopenia | ||
650 | 4 | |a Journal Article | |
650 | 4 | |a Research Support, Non-U.S. Gov't | |
650 | 4 | |a Review | |
650 | 4 | |a Inherited thrombocytopenia | |
650 | 4 | |a bleeding disorders | |
650 | 4 | |a germline predisposition | |
650 | 4 | |a platelet disorders | |
650 | 4 | |a platelet dysfunction | |
650 | 4 | |a thrombopoietin-receptor agonists | |
700 | 1 | |a Balduini, Carlo L |e verfasserin |4 aut | |
773 | 0 | 8 | |i Enthalten in |t Blood reviews |d 1995 |g 48(2021) vom: 15. Juli, Seite 100784 |w (DE-627)NLM013343343 |x 1532-1681 |7 nnns |
773 | 1 | 8 | |g volume:48 |g year:2021 |g day:15 |g month:07 |g pages:100784 |
856 | 4 | 0 | |u http://dx.doi.org/10.1016/j.blre.2020.100784 |3 Volltext |
912 | |a GBV_USEFLAG_A | ||
912 | |a GBV_NLM | ||
951 | |a AR | ||
952 | |d 48 |j 2021 |b 15 |c 07 |h 100784 |