The term 'sideroblastic anemia' (SA) encompasses a diverse collection of diseases in which different causes and different mechanisms converge to produce the same rather spectacular feature: the accumulation of inorganic iron in the cytoplasm of erythroid cells in sufficient quantities to be easily demonstrated in the form of granules by using Perl's Prussian Blue staining. Characteristically, the iron is found in mitochondria positioned around the nucleus — hence the term 'ring sid-eroblast'. SAs are broadly divided into acquired and inherited forms (Table 12.3). Acquired SAs are the most common and are discussed in Chapter 8. Here, we discuss briefly the three inherited forms of SA for which the genetic and biochemical bases have been elucidated.
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