Refractory anemia with ringed sideroblasts associated with thrombocytosis: comparative analysis of marked with non-marked thrombocytosis, and relationship with JAK2 V617F mutational status.
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.
Raya
J M
JM
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain. jomaraya@yahoo.es.
Arenillas
L
L
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Domingo
A
A
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Bellosillo
B
B
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Gutiérrez
G
G
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Luño
E
E
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Piñán
M A
MA
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Barbón
M
M
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Pérez-Sirvent
M L
ML
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Muruzábal
M J
MJ
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Yánez
L
L
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
García
L
L
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Lemes
A
A
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Navarro
J T
JT
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Elosegi
A
A
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Cortés
M A
MA
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Villegas
A
A
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Durán
M A
MA
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Ardanaz
M
M
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
Florensa
L
L
Servicio de Hematología y Hemoterapia, Hospital Universitario de Canarias, Ofra, s/n, 38320, La Laguna, Tenerife, Spain.
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019943:Amino Acid Substitution; D000753:Anemia, Refractory / Q000097:blood / Q000235:genetics* / Q000473:pathology*; D001854:Bone Marrow Cells / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D010976:Platelet Count; D012189:Retrospective Studies; D013922:Thrombocytosis / Q000097:blood / Q000235:genetics* / Q000473:pathology*
C507924:JAK2 protein, human; D053614:Janus Kinase 2