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Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis.
- Source :
-
Haematologica [Haematologica] 2012 Jul; Vol. 97 (7), pp. 1036-41. Date of Electronic Publication: 2012 Apr 24. - Publication Year :
- 2012
-
Abstract
- Background: Refractory anemia with ring sideroblasts associated with marked thrombocytosis was proposed as a provisional entity in the 2001 World Health Organization classification of myeloid neoplasms and also in the 2008 version, but its existence as a single entity is contested. We wish to define the clinical features of this rare myelodysplastic/myeloproliferative neoplasm and to compare its clinical outcome with that of refractory anemia with ring sideroblasts and essential thrombocythemia.<br />Design and Methods: We conducted a collaborative retrospective study across Europe. Our database included 200 patients diagnosed with refractory anemia with ring sideroblasts and marked thrombocytosis. For each of these patients, each patient diagnosed with refractory anemia with ring sideroblasts was matched for age and sex. At the same time, a cohort of 454 patients with essential thrombocythemia was used to compare outcomes of the two diseases.<br />Results: In patients with refractory anemia with ring sideroblasts and marked thrombocytosis, depending on the Janus Kinase 2 V617F mutational status (positive or negative) or platelet threshold (over or below 600 × 10(9)/L), no difference in survival was noted. However, these patients had shorter overall survival and leukemia-free survival with a lower risk of thrombotic complications than did patients with essential thrombocythemia (P<0.001) but better survival (P<0.001) and a higher risk of thrombosis (P=0.039) than patients with refractory anemia with ring sideroblasts.<br />Conclusions: The clinical course of refractory anemia with ring sideroblasts and marked thrombocytosis is better than that of refractory anemia with ring sideroblasts and worse than that of essential thrombocythemia. The higher risk of thrombotic events in this disorder suggests that anti-platelet therapy might be considered in this subset of patients. From a clinical point of view, it appears to be important to consider refractory anemia with ring sideroblasts and marked thrombocytosis as a distinct entity.
- Subjects :
- Adolescent
Adult
Aged
Aged, 80 and over
Anemia, Refractory complications
Anemia, Refractory mortality
Anemia, Sideroblastic complications
Anemia, Sideroblastic mortality
Blood Platelets pathology
Europe
Female
Humans
Male
Middle Aged
Mutation
Platelet Count
Retrospective Studies
Risk Factors
Survival Analysis
Thrombocythemia, Essential complications
Thrombocythemia, Essential mortality
Thrombocytosis complications
Thrombocytosis mortality
Anemia, Refractory pathology
Anemia, Sideroblastic pathology
Janus Kinase 2 genetics
Thrombocythemia, Essential pathology
Thrombocytosis pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1592-8721
- Volume :
- 97
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Haematologica
- Publication Type :
- Academic Journal
- Accession number :
- 22532522
- Full Text :
- https://doi.org/10.3324/haematol.2011.053918