Outcome of Refractory Anemia with Ringed Sideroblasts Associated with Marked Thrombocytosis (RARS-T) In a Large Cohort of Patients
| Autor: | Mario Cazzola, Esther Zipperer, Sylvie Hermouet, Susanne Schnittger, Lourdes Florensa, Julien Broséus, Marc Maynadié, Jyoti Nangalia, Morgane Mounier, Carlos Besses, Steven Richebourg, Torsten Haferlach, Eric Lippert, François Girodon, José María Raya, Norbert Gattermann, Claudia Haferlach, Erica Travaglino, Ulrich Germing, Luca Malcovati, Allou Kaoutar, Jaroslav Cermak, Richard Garand |
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| Rok vydání: | 2010 |
| Předmět: |
Pediatrics
medicine.medical_specialty Thrombocytosis business.industry Essential thrombocythemia organic chemicals Myelodysplastic syndromes Immunology Cell Biology Hematology Refractory anemia with ringed sideroblasts medicine.disease Biochemistry Gastroenterology biological factors body regions Leukemia Myeloproliferative Disorders Dysplasia Internal medicine embryonic structures medicine Hemoglobin business neoplasms |
| Zdroj: | Blood. 116:4113-4113 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v116.21.4113.4113 |
| Popis: | Abstract 4113 Introduction: Most of the data related to RARS-T, a rare disorder, involve small cohorts of patients. We aimed to analyze more patients also considering a variety of myelodysplastic or myeloproliferative disorders. Objective: To compare a large cohort of patients with RARS-T to refractory anemia with ringed sideroblasts (RARS), refractory anemia with ringed sideroblasts and multilineage dysplasia (RARS-MD) or essential thrombocythemia (ET) at the time of diagnosis and during disease evolution, in terms of survival and complications. Materials: Data of a European multi-center study was used including 199 cases of RARS-T 173 cases of RARS, 102 cases of RARS-MD and 431 cases of ET. Results: At baseline, compared to RARS and RARS-MD patients, RARS-T patients had similar hemoglobin concentration, but a higher white blood count. The JAK2V617F mutation was observed in 43%, 12% and 5% in RARS-T, RARS and RARS-MD patients, respectively. When separated in 2 groups (450,000600,000 × 109/l), RARS-T patients were comparable for sex, age, hemoglobin level and survival. However, patients with platelet count > 600,000 × 109/l had higher WBC (11 ×109/l versus 7.5 ×109/l, p Conclusion: According to our results, the outcome in RARS-T more closely mimics myelodysplastic syndromes rather than myeloproliferative neoplasms. Our results agree with the WHO 2008 classification that considers RARS-T as a separate disorder. Disclosures: Schnittger: MLL Munich Leukemia Laboratory: Employment, Equity Ownership. Gattermann:Novartis: Honoraria, Research Funding. Haferlach:MLL Munich Leukemia Laboratory: Employment, Equity Ownership. |
| Databáze: | OpenAIRE |
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