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Article: Refractory anemia with ringed sideroblasts in children: Two diseases with a similar phenotype?

TitleRefractory anemia with ringed sideroblasts in children: Two diseases with a similar phenotype?
Authors
Issue Date1999
PublisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.jpho-online.com
Citation
Journal Of Pediatric Hematology/Oncology, 1999, v. 21 n. 5, p. 418-423 How to Cite?
AbstractThree pediatric patients with refractory anemia with ringed sideroblasts (RARS) are presented. Bone marrow aspirates were examined using Romanowsky and Prussian blue iron stains in all three patients, and electron microscopic analysis was performed in one patient. All three patients had cytogenetic analysis of the bone marrow. Other studies included analysis of serum iron, total iron-binding capacity, ferritin, copper, vitamins B6 and B12, and folate levels. Antibody titers to Parvovirus, HIV, and other viruses were measured. The patients had contrasting clinical courses. Patients 1 rand 2 had dysplastic hematopoietic features and cytogenetic findings (with either partial or one allele loss of chromosome 7), suggestive of myelodysplastic syndrome. Patient 1 experienced acute myeloid leukemia (AML) and had a good response to AML-directed therapy. Patient 2 had prolonged cytopenias and underwent bone marrow transplantation (BMT). Patient 3 had features suggestive of refractory anemia associated with mitochondrial cytopathy, including normal cytogenetics with pronounced vacuolization of marrow precursors. His anemia regressed spontaneously a few months after diagnosis. These patients represent two subgroups of pediatric RARS. Patients with the myelodysplastic syndrome (MDS) type may progress to cytopenias or leukemia and may require aggressive therapy; the type is characterized by clonal cytogenetic findings. The non-MDS type, which may relate to mitochondrial cytopathy, often shows spontaneous regression and requires only supportive treatment; it has normal cytogenetic findings.
Persistent Identifierhttp://hdl.handle.net/10722/79835
ISSN
2015 Impact Factor: 1.146
2015 SCImago Journal Rankings: 0.452
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChan, GCFen_HK
dc.contributor.authorHead, DRen_HK
dc.contributor.authorWang, WCen_HK
dc.date.accessioned2010-09-06T07:59:17Z-
dc.date.available2010-09-06T07:59:17Z-
dc.date.issued1999en_HK
dc.identifier.citationJournal Of Pediatric Hematology/Oncology, 1999, v. 21 n. 5, p. 418-423en_HK
dc.identifier.issn1077-4114en_HK
dc.identifier.urihttp://hdl.handle.net/10722/79835-
dc.description.abstractThree pediatric patients with refractory anemia with ringed sideroblasts (RARS) are presented. Bone marrow aspirates were examined using Romanowsky and Prussian blue iron stains in all three patients, and electron microscopic analysis was performed in one patient. All three patients had cytogenetic analysis of the bone marrow. Other studies included analysis of serum iron, total iron-binding capacity, ferritin, copper, vitamins B6 and B12, and folate levels. Antibody titers to Parvovirus, HIV, and other viruses were measured. The patients had contrasting clinical courses. Patients 1 rand 2 had dysplastic hematopoietic features and cytogenetic findings (with either partial or one allele loss of chromosome 7), suggestive of myelodysplastic syndrome. Patient 1 experienced acute myeloid leukemia (AML) and had a good response to AML-directed therapy. Patient 2 had prolonged cytopenias and underwent bone marrow transplantation (BMT). Patient 3 had features suggestive of refractory anemia associated with mitochondrial cytopathy, including normal cytogenetics with pronounced vacuolization of marrow precursors. His anemia regressed spontaneously a few months after diagnosis. These patients represent two subgroups of pediatric RARS. Patients with the myelodysplastic syndrome (MDS) type may progress to cytopenias or leukemia and may require aggressive therapy; the type is characterized by clonal cytogenetic findings. The non-MDS type, which may relate to mitochondrial cytopathy, often shows spontaneous regression and requires only supportive treatment; it has normal cytogenetic findings.en_HK
dc.languageengen_HK
dc.publisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.jpho-online.comen_HK
dc.relation.ispartofJournal of Pediatric Hematology/Oncologyen_HK
dc.rightsJournal of Pediatric Hematology / Oncology. Copyright © Lippincott Williams & Wilkins.en_HK
dc.subject.meshAdolescenten_HK
dc.subject.meshAnemia, Refractory, with Excess of Blasts - genetics - pathologyen_HK
dc.subject.meshBone Marrow Cells - pathologyen_HK
dc.subject.meshBone Marrow Transplantationen_HK
dc.subject.meshChilden_HK
dc.subject.meshChromosomes, Human, Pair 7en_HK
dc.subject.meshErythroblasts - pathology - ultrastructureen_HK
dc.subject.meshFemaleen_HK
dc.subject.meshHumansen_HK
dc.subject.meshIron - analysisen_HK
dc.subject.meshLeukemia, Myeloid, Acute - complications - therapyen_HK
dc.subject.meshLoss of Heterozygosityen_HK
dc.subject.meshMaleen_HK
dc.subject.meshMitochondria - pathology - ultrastructureen_HK
dc.subject.meshPhenotypeen_HK
dc.titleRefractory anemia with ringed sideroblasts in children: Two diseases with a similar phenotype?en_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1077-4114&volume=21&issue=5&spage=419&epage=424&date=1999&atitle=Refractory+Anemia+With+Ringed+Sideroblasts+in+Children:+Two+Diseases+With+a+Similar+Phenotype?en_HK
dc.identifier.emailChan, GCF:gcfchan@hkucc.hku.hken_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1097/00043426-199909000-00015en_HK
dc.identifier.pmid10524457-
dc.identifier.scopuseid_2-s2.0-0033496716en_HK
dc.identifier.hkuros47417en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0033496716&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume21en_HK
dc.identifier.issue5en_HK
dc.identifier.spage418en_HK
dc.identifier.epage423en_HK
dc.identifier.isiWOS:000082935400016-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK
dc.identifier.scopusauthoridHead, DR=7102355559en_HK
dc.identifier.scopusauthoridWang, WC=7501758774en_HK

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