Is refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T) a necessary or useful diagnostic category?
Both the 2001 World Health Organisation (WHO) classification of haematopoietic neoplasms and the 2008 WHO classification revision include a distinctive diagnostic category, refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T), to describe those rare patients who have both >or=15% ring sideroblasts and a sustained elevated platelet count. Recently, it has become clear that patients meeting WHO criteria for RARS-T have clonal JAK2(V617F) and MPL(W515) mutations at a similar rate to essential thrombocythaemia (ET). Given that the provisional classification of RARS-T as a myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) overlap syndrome, rather than as a form of MPN (i.e., ET), rests principally upon the presence of ring sideroblasts, which are a non-specific morphological finding, these new molecular results prompt reconsideration of the necessity for a distinctive RARS-T category. Here we review the historical developments that led up the definition of RARS-T as a disease entity, and we discuss conceptual understanding of RARS-T and arguments against continued use of RARS-T as a separate diagnostic category.
Wardrop
Douglas
D
Oxford Haemophilia and Thrombosis Centre, Churchill Hospital, University of Oxford, Oxford, UK.
Steensma
David P
DP
D000753:Anemia, Refractory / Q000145:classification* / Q000175:diagnosis / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D009154:Mutation; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000235:genetics; D013926:Thrombopoietin / Q000235:genetics; D014944:World Health Organization
D013926:Thrombopoietin; D053614:Janus Kinase 2