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Refractory anemia with ringed sideroblasts associated with thrombocytosis: comparative analysis of marked with non-marked thrombocytosis, and relationship with JAK2 V617F mutational status

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Abstract

The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues (2001) defined a provisional entity named refractory anemia with ringed sideroblasts associated to marked thrombocytosis (RARS-MT). Diagnosis of RARS-MT requires more than 15% of ringed sideroblasts in bone marrow aspirate and the existence of a thrombocytosis in blood, with a platelet count above 600 × 109/L. Nevertheless, controversy exists regarding this platelet count “cut-off” value and, when RARS-MT was defined, the JAK2 mutation and its importance in the study of myeloproliferative disorders was unknown. We present the results of a Spanish retrospective multicentric study, which includes 76 cases of RARS with associated thrombocytosis (platelet count above 400 × 109/L) at diagnosis (RARS-T), 36 of them with a platelet count above 600 × 109/L. Our aim was to analyze their clinical, analytical and morphological characteristics, and to establish correlations with the JAK2 mutational status.

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Aknowledgments

This work has been partially supported by a FIS grant (PI030345) and a FEHH grant (Fundación Española de Hematología y Hemoterapia). Spanish Hospitals, Cities And Doctors′ Name: H. del Mar, Barcelona (L Florensa, L Arenillas, B Bellosillo, S Woessner); H. Universitari de Bellvitge, Hospitalet, Barcelona (A Domingo, E Alonso); H. Clinic i Provincial, Barcelona (M Rozman, G Gutiérrez); H. Central de Asturias (E Luño, C Sanzo); H. de Cruces, Baracaldo, Vizcaya (MA Piñán, G Letamendi); H. La Fe, Valencia (ML Perez-Sirvent, J Cervera); H. de León, León (M Barbón); H. de Getafe, Madrid (L García); H. Doctor Negrín, Las Palmas (A Lemes, T Molero); H. Marqués de Valdecilla, Santander (L Yáñez); H. Sierrallana, Torrelavega, Cantabria (JI Olalla, MJ Muruzabal); H. Germans, Trias y Pujol, Badalona, Barcelona (F Millá, JT Navarro); H. Txagorritxu, Vitoria (M Ardanaz); H. de Zumárraga, Guipúzcoa (A Elosegui, T Hernandez-Santamaría); H. Clínico San Carlos, Madrid (A Villegas, M Mateo); H. de Laredo, Cantabria (MA Cortés, ML González-Ponte); H. Son Dureta, Palma de Mallorca (MA Durán); H. Vall d′Hebrón (T Vallespí); and H. Universitario de Canarias, La Laguna (JM Raya, T Martín, L Morabito, L Hernández-Nieto).

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Authors and Affiliations

  1. Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain

    J. M. Raya, L. Arenillas, A. Domingo, B. Bellosillo, G. Gutiérrez, E. Luño, M. A. Piñán, M. Barbón, M. L. Pérez-Sirvent, M. J. Muruzábal, L. Yánez, L. García, A. Lemes, J. T. Navarro, A. Elosegi, M. A. Cortés, A. Villegas, M. A. Durán, M. Ardanaz & L. Florensa

Authors
  1. J. M. Raya
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  2. L. Arenillas
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  3. A. Domingo
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  4. B. Bellosillo
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  5. G. Gutiérrez
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  6. E. Luño
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  7. M. A. Piñán
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  8. M. Barbón
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  9. M. L. Pérez-Sirvent
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  10. M. J. Muruzábal
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  11. L. Yánez
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  12. L. García
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  13. A. Lemes
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  14. J. T. Navarro
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  15. A. Elosegi
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  16. M. A. Cortés
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  17. A. Villegas
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  18. M. A. Durán
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  19. M. Ardanaz
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  20. L. Florensa
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On behalf of the Grupo Español de Citología Hematológica (GECH), Working Group into the Asociación Española de Hematología y Hemoterapia (AEHH)

Corresponding author

Correspondence to J. M. Raya.

Additional information

This work is multicentric, with data collected and centralized at Hospital Universitario de Canarias (La Laguna, Spain).

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Raya, J.M., Arenillas, L., Domingo, A. et al. Refractory anemia with ringed sideroblasts associated with thrombocytosis: comparative analysis of marked with non-marked thrombocytosis, and relationship with JAK2 V617F mutational status. Int J Hematol 88, 387–395 (2008). https://doi.org/10.1007/s12185-008-0169-1

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  • DOI: https://doi.org/10.1007/s12185-008-0169-1

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