Matches in SemOpenAlex for { <https://semopenalex.org/work/W3088209099> ?p ?o ?g. }
- W3088209099 endingPage "318" @default.
- W3088209099 startingPage "305" @default.
- W3088209099 abstract "Sideroblastic anemia (SA) consists of a group of inherited and acquired anemias of ineffective erythropoiesis characterized by the accumulation of ring sideroblasts in the bone marrow due to disrupted heme biosynthesis. Congenital sideroblastic anemia (CSA) is rare and has three modes of inheritance: X-linked (XLSA), autosomal recessive (ARCSA), and maternal. Acquired SA is more common and can be a result of myelodysplastic syndromes (MDS) or other, generally reversible causes. The diagnostic approach to SA includes a work-up for reversible causes and genetic testing for CSA based on clinical suspicion, family history and genetic pedigree. The treatment of SA depends on the underlying etiology but remains primarily supportive with vitamin B6 supplementation for select cases of XLSA, thiamine for thiamine-responsive megaloblastic anemia subtype, red blood cell transfusions for symptomatic patients and iron chelation therapy for iron overload. The management of anemia in MDS subtypes with ring sideroblasts remains unique and includes the recently approved erythroid maturation agent, Luspatercept. Although there is currently no curative therapy for CSA, anecdotal reports of hematopoietic stem cell transplant demonstrate remissions in selective, non-syndromic cases. This review summarizes the genetics, pathophysiology, diagnosis and treatment of SA for general practitioners and clinical hematologists." @default.
- W3088209099 created "2020-10-01" @default.
- W3088209099 creator A5075145540 @default.
- W3088209099 creator A5078927879 @default.
- W3088209099 date "2020-09-01" @default.
- W3088209099 modified "2023-10-16" @default.
- W3088209099 title "<p>Understanding Sideroblastic Anemia: An Overview of Genetics, Epidemiology, Pathophysiology and Current Therapeutic Options</p>" @default.
- W3088209099 cites W151933253 @default.
- W3088209099 cites W1704670422 @default.
- W3088209099 cites W185078569 @default.
- W3088209099 cites W1889574318 @default.
- W3088209099 cites W1917604757 @default.
- W3088209099 cites W1965134802 @default.
- W3088209099 cites W1972547633 @default.
- W3088209099 cites W1977142594 @default.
- W3088209099 cites W1981093010 @default.
- W3088209099 cites W1981801074 @default.
- W3088209099 cites W1986018588 @default.
- W3088209099 cites W1987860556 @default.
- W3088209099 cites W1991907049 @default.
- W3088209099 cites W1992193197 @default.
- W3088209099 cites W1992803617 @default.
- W3088209099 cites W1996982923 @default.
- W3088209099 cites W1999982374 @default.
- W3088209099 cites W2012930099 @default.
- W3088209099 cites W2013232197 @default.
- W3088209099 cites W2013995411 @default.
- W3088209099 cites W2021333892 @default.
- W3088209099 cites W2032628875 @default.
- W3088209099 cites W2039661759 @default.
- W3088209099 cites W2045930137 @default.
- W3088209099 cites W2054024401 @default.
- W3088209099 cites W2055828466 @default.
- W3088209099 cites W2059646125 @default.
- W3088209099 cites W2062686654 @default.
- W3088209099 cites W2063509140 @default.
- W3088209099 cites W2067282039 @default.
- W3088209099 cites W2069752681 @default.
- W3088209099 cites W2072198901 @default.
- W3088209099 cites W2075352689 @default.
- W3088209099 cites W2076739425 @default.
- W3088209099 cites W2079314569 @default.
- W3088209099 cites W2080762011 @default.
- W3088209099 cites W2088578052 @default.
- W3088209099 cites W2095232221 @default.
- W3088209099 cites W2097252258 @default.
- W3088209099 cites W2103199749 @default.
- W3088209099 cites W2114400437 @default.
- W3088209099 cites W2136593078 @default.
- W3088209099 cites W2144892629 @default.
- W3088209099 cites W2146155744 @default.
- W3088209099 cites W2148216967 @default.
- W3088209099 cites W2155113724 @default.
- W3088209099 cites W2309192305 @default.
- W3088209099 cites W2319920590 @default.
- W3088209099 cites W2339658711 @default.
- W3088209099 cites W2414573452 @default.
- W3088209099 cites W2463012969 @default.
- W3088209099 cites W2463506112 @default.
- W3088209099 cites W2470928828 @default.
- W3088209099 cites W2480485646 @default.
- W3088209099 cites W2586125700 @default.
- W3088209099 cites W2749855858 @default.
- W3088209099 cites W2760847166 @default.
- W3088209099 cites W2768612073 @default.
- W3088209099 cites W28713408 @default.
- W3088209099 cites W2885795181 @default.
- W3088209099 cites W2906690257 @default.
- W3088209099 cites W2998901800 @default.
- W3088209099 cites W4239836009 @default.
- W3088209099 cites W4246294583 @default.
- W3088209099 cites W4246317953 @default.
- W3088209099 cites W71515697 @default.
- W3088209099 cites W4251155103 @default.
- W3088209099 doi "https://doi.org/10.2147/jbm.s232644" @default.
- W3088209099 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7524202" @default.
- W3088209099 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33061728" @default.
- W3088209099 hasPublicationYear "2020" @default.
- W3088209099 type Work @default.
- W3088209099 sameAs 3088209099 @default.
- W3088209099 citedByCount "19" @default.
- W3088209099 countsByYear W30882090992021 @default.
- W3088209099 countsByYear W30882090992022 @default.
- W3088209099 countsByYear W30882090992023 @default.
- W3088209099 crossrefType "journal-article" @default.
- W3088209099 hasAuthorship W3088209099A5075145540 @default.
- W3088209099 hasAuthorship W3088209099A5078927879 @default.
- W3088209099 hasBestOaLocation W30882090991 @default.
- W3088209099 hasConcept C10162356 @default.
- W3088209099 hasConcept C126322002 @default.
- W3088209099 hasConcept C137627325 @default.
- W3088209099 hasConcept C187212893 @default.
- W3088209099 hasConcept C2778248108 @default.
- W3088209099 hasConcept C2779188306 @default.
- W3088209099 hasConcept C2779703530 @default.
- W3088209099 hasConcept C2780007613 @default.
- W3088209099 hasConcept C2780817109 @default.
- W3088209099 hasConcept C2780844101 @default.