Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20 % of cases. Among them, immune thrombocytopenia (ITP) has been reported but large studies assessing this association missing. Whether such patients have a particular phenotype require management is unclear.
 This study analyzes the clinical spectrum, outcome therapeutic ITP MDS CMML, comparison (i) to primary without MDS/CMML (ii) ITP.
 Forty-one MDS/CMML-associated were included, 26 (63%) patients, low-risk myelodysplasia 30 (73%) CMML 24 (59%) patients. An disease was noted 10 (24%) In had higher occurrence severe bleeding despite similar platelet counts at diagnosis. First-line treatment consisted glucocorticoids (98%) intravenous immunoglobulin (IVIg) (56%). Response achievement IVIg more frequent than rates second-line therapies not statistically different between MDS/CMMLassociated Ten percent (n=4) multirefractory versus none controls. After median follow-up 60 months, there no difference overall survival Leukemia-free-survival significantly better profile ITP, response therapy except for IVIg, less progression toward acute myeloid ITP.