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International Journal of Hematology

, Volume 88, Issue 4, pp 387–395 | Cite as

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

  • J. M. RayaEmail author
  • 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
  • On behalf of the Grupo Español de Citología Hematológica (GECH), Working Group into the Asociación Española de Hematología y Hemoterapia (AEHH)
Original Article

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.

Keywords

Refractory anemia Ringed sideroblasts Thrombocytosis JAK2 mutation 

Notes

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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Copyright information

© The Japanese Society of Hematology 2008

Authors and Affiliations

  • J. M. Raya
    • 1
    Email author
  • L. Arenillas
    • 1
  • A. Domingo
    • 1
  • B. Bellosillo
    • 1
  • G. Gutiérrez
    • 1
  • E. Luño
    • 1
  • M. A. Piñán
    • 1
  • M. Barbón
    • 1
  • M. L. Pérez-Sirvent
    • 1
  • M. J. Muruzábal
    • 1
  • L. Yánez
    • 1
  • L. García
    • 1
  • A. Lemes
    • 1
  • J. T. Navarro
    • 1
  • A. Elosegi
    • 1
  • M. A. Cortés
    • 1
  • A. Villegas
    • 1
  • M. A. Durán
    • 1
  • M. Ardanaz
    • 1
  • L. Florensa
    • 1
  • On behalf of the Grupo Español de Citología Hematológica (GECH), Working Group into the Asociación Española de Hematología y Hemoterapia (AEHH)
  1. 1.Servicio de Hematología y HemoterapiaHospital Universitario de CanariasLa Laguna, TenerifeSpain

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