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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J. T. Navarro, L. García, A. Lemes, M. A. Durán, M. A. Cortés, Lucrecia Yañez, A. Domingo, J. M. Raya, M. Ardanaz, M. J. Muruzábal, A. Elosegi, María Ángeles Piñan, Maria Luz Perez-Sirvent, Leonor Arenillas, A. Villegas, B. Bellosillo, Elisa Luño, L. Florensa, M. Barbón, and G. Gutiérrez
- Subjects
Adult ,Male ,Pathology ,medicine.medical_specialty ,Mutation, Missense ,Bone Marrow Cells ,Refractory anemia with ringed sideroblasts ,World health ,Bone marrow aspirate ,Myeloproliferative Disorders ,medicine ,Mutational status ,Humans ,Platelet ,Aged ,Retrospective Studies ,Aged, 80 and over ,Thrombocytosis ,business.industry ,Platelet Count ,Anemia, Refractory ,Hematology ,Janus Kinase 2 ,Middle Aged ,medicine.disease ,Amino Acid Substitution ,embryonic structures ,Female ,business ,JAK2 V617F - 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