ABSTRACT
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 are missing. Whether such patients have a particular phenotype and require particular management is unclear. This study analyzes the clinical spectrum, outcome and therapeutic management of patients with ITP associated with MDS or CMML, in comparison (i) to patients with primary ITP without MDS/CMML and (ii) to patients with MDS/CMML without ITP. Forty-one MDS/CMML-associated ITP patients were included, with chronic ITP in 26 (63%) patients, low-risk myelodysplasia in 30 (73%) patients and CMML in 24 (59%) patients. An associated autoimmune disease was noted in 10 (24%) patients. In comparison to primary ITP patients, MDS/CMML-associated ITP patients had a higher occurrence of severe bleeding despite similar platelet counts at diagnosis. First-line treatment consisted of glucocorticoids (98%) and intravenous immunoglobulin (IVIg) (56%). Response achievement with IVIg was more frequent in primary ITP than in MDS/CMML-associated ITP patients. Response rates to second-line therapies were not statistically different between primary ITP and MDS/CMMLassociated ITP patients. Ten percent (n=4) of patients with MDS/CMML-associated ITP had multirefractory ITP versus none in primary ITP controls. After a median follow-up of 60 months, there was no difference in overall survival between MDS/CMML-associated ITP and primary ITP patients. Leukemia-free-survival was significantly better in MDS/CMMLassociated ITP patients than in MDS/CMML without ITP MDS/CMML-associated ITP have a particular outcome with more severe bleeding and multirefractory profile than primary ITP, similar response profile to primary ITP therapy except for IVIg, and less progression toward acute myeloid leukemia than MDS/CMML without ITP.
Subject(s)
Leukemia, Myeloid, Acute , Leukemia, Myelomonocytic, Chronic , Myelodysplastic Syndromes , Purpura, Thrombocytopenic, Idiopathic , Thrombocytopenia , Humans , Leukemia, Myelomonocytic, Chronic/complications , Leukemia, Myelomonocytic, Chronic/diagnosis , Leukemia, Myelomonocytic, Chronic/therapy , Myelodysplastic Syndromes/complications , Myelodysplastic Syndromes/diagnosis , Myelodysplastic Syndromes/therapy , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/etiology , Purpura, Thrombocytopenic, Idiopathic/therapyABSTRACT
For unknown reasons, individuals who are confident in their intuitions are more likely to hold supernatural beliefs. How does an intuitive cognitive style lead one to believe in faith healing, astrology, or extrasensory perception (ESP)? We hypothesize that cognitive style is critically important after one experiences an uncanny event that seems to invite a supernatural explanation. In three studies, we show that irrespective of their prior beliefs in the supernatural, non-reflective thinkers are more likely than reflective thinkers to accept supernatural causation after an uncanny encounter with astrology and ESP. This is the first time that controlled experiments demonstrate the negative dynamics of reflection and supernatural causality attribution. We consider the possible generalization of our findings to religious beliefs and their implications for the social vulnerability of non-reflective individuals.
