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Is refractory anaemia with ring sideroblasts and thrombocytosis (RARS‐T) a necessary or useful diagnostic category?
Author(s) -
Wardrop Douglas,
Steensma David P.
Publication year - 2009
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2008.07526.x
Subject(s) - thrombocytosis , myeloproliferative neoplasm , medicine , disease entity , myeloproliferative disorders , disease , platelet , myelofibrosis , bone marrow
Summary 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 ≥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.