Source:http://linkedlifedata.com/resource/pubmed/id/21350094
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
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pubmed:dateCreated |
2011-2-25
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pubmed:abstractText |
Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a provisional entity in the current World Health Organization classification and is thought to be a myelodysplastic/myeloproliferative neoplasm (MDS/MPN). We analyzed 18 cases of RARS-T. All patients had thrombocytosis (platelet count, 515-1,100 × 10(3)/?L [515-1,100 × 10(9)/L]) and anemia (hemoglobin level, 7.2-12.6 g/dL [72-126 g/L]). Three patients had mild leukocytosis (WBC count, 3,900-16,300/?L [3.9-16.3 × 10(9)/L]). Ring sideroblasts were 8% to 75% in the bone marrow. Megakaryocytes showed a spectrum of morphologic findings. JAK2(V617F) was identified in 9 of 15 cases, including 7 of 9 with thrombocytosis (platelet count, >600 × 10(3)/?L [600 × 10(9)/L]) and 1 with 8% ring sideroblasts. The MPL(W515L) mutation was not detected (n = 9). We conclude that RARS-T is a pathogenetically heterogeneous group of limited diagnostic usefulness. Approximately 60% of cases carry JAK2(V617F)and seem to be closer to an MPN in which ring sideroblasts may be a secondary phenomenon. The remaining cases usually lack the JAK2(V617F)mutation, have a platelet count less than 600 × 10(3)/?L (600 × 10(9)/L), and may represent an MDS or MPN with thrombocytosis of unknown mechanisms.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
1943-7722
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pubmed:author | |
pubmed:issnType |
Electronic
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pubmed:volume |
135
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
398-403
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pubmed:meshHeading |
pubmed-meshheading:21350094-Adult,
pubmed-meshheading:21350094-Aged,
pubmed-meshheading:21350094-Aged, 80 and over,
pubmed-meshheading:21350094-Anemia, Refractory,
pubmed-meshheading:21350094-Anemia, Sideroblastic,
pubmed-meshheading:21350094-Bone Marrow Cells,
pubmed-meshheading:21350094-Female,
pubmed-meshheading:21350094-Humans,
pubmed-meshheading:21350094-Janus Kinase 2,
pubmed-meshheading:21350094-Leukocytosis,
pubmed-meshheading:21350094-Male,
pubmed-meshheading:21350094-Middle Aged,
pubmed-meshheading:21350094-Mutation,
pubmed-meshheading:21350094-Retrospective Studies,
pubmed-meshheading:21350094-Thrombocytosis
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pubmed:year |
2011
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pubmed:articleTitle |
Refractory anemia with ring sideroblasts associated with marked thrombocytosis: a mixed group exhibiting a spectrum of morphologic findings.
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pubmed:affiliation |
Department of Hematopathology, The University of Texas M. D. Anderson Cancer Center, Houston, 77030, USA.
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pubmed:publicationType |
Journal Article
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