1. Refractory anemia with ringed sideroblasts associated with thrombocytosis: comparative analysis of marked with non-marked thrombocytosis, and relationship with JAK2 V617F mutational status.
- Author
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Raya JM, Arenillas L, Domingo A, Bellosillo B, Gutiérrez G, Luño E, Piñán MA, Barbón M, Pérez-Sirvent ML, Muruzábal MJ, Yánez L, García L, Lemes A, Navarro JT, Elosegi A, Cortés MA, Villegas A, Durán MA, Ardanaz M, and Florensa L
- Subjects
- Adult, Aged, Aged, 80 and over, Amino Acid Substitution, Anemia, Refractory blood, Bone Marrow Cells metabolism, Bone Marrow Cells pathology, Female, Humans, Janus Kinase 2 metabolism, Male, Middle Aged, Platelet Count, Retrospective Studies, Thrombocytosis blood, Anemia, Refractory genetics, Anemia, Refractory pathology, Janus Kinase 2 genetics, Mutation, Missense, Thrombocytosis genetics, Thrombocytosis pathology
- 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 x 10(9)/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 x 10(9)/L) at diagnosis (RARS-T), 36 of them with a platelet count above 600 x 10(9)/L. Our aim was to analyze their clinical, analytical and morphological characteristics, and to establish correlations with the JAK2 mutational status.
- Published
- 2008
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