Deletion of the long arm of chromosome 20—del(20q)—is commonly associated with myeloid malignancies, in particular with myeloproliferative disorders (MPD), myelodysplastic syndrome (MDS), and acute ...myelogenous leukemia (AML). Its association with the hypereosinophilic syndrome (HES) had never been reported. In the present study we describe two patients with long-standing hypereosinophilia and features of atypical MPD or MDS carrying a del(20q) as a constant and isolated chromosomal abnormality. One patient, with an aggressive clinical course, died within 2 years, despite several lines of treatment. The other patient had a more indolent course consistent with that of an atypical MDS with eosinophilia.
Summary
Background
Cardiac involvement is a major cause of mortality in patients with thrombotic thrombocytopenic purpura (TTP). However, diagnosis remains underestimated and delayed, owing to ...subclinical injuries. Cardiac troponin‐I measurement (cTnI) on admission could improve the early diagnosis of cardiac involvement and have prognostic value.
Objectives
To assess the predictive value of cTnI in patients with TTP for death or refractoriness.
Patients/Methods
The study involved a prospective cohort of adult TTP patients with acquired severe ADAMTS‐13 deficiency (< 10%) and included in the registry of the French Reference Center for Thrombotic Microangiopathies. Centralized cTnI measurements were performed on frozen serum on admission.
Results
Between January 2003 and December 2011, 133 patients with TTP (mean age, 48 ± 17 years) had available cTnI measurements on admission. Thirty‐two patients (24%) had clinical and/or electrocardiogram features. Nineteen (14.3%) had cardiac symptoms, mainly congestive heart failure and myocardial infarction. Electrocardiogram changes, mainly repolarization disorders, were present in 13 cases. An increased cTnI level (> 0.1 μg L−1) was present in 78 patients (59%), of whom 46 (59%) had no clinical cardiac involvement. The main outcomes were death (25%) and refractoriness (17%). Age (P = 0.02) and cTnI level (P = 0.002) showed the greatest impact on survival. A cTnI level of > 0.25 μg L−1 was the only independent factor in predicting death (odds ratio OR 2.87; 95% confidence interval CI 1.13–7.22; P = 0.024) and/or refractoriness (OR 3.03; 95% CI 1.27–7.3; P = 0.01).
Conclusions
A CTnI level of > 0.25 μg L−1 at presentation in patients with TTP appears to be an independent factor associated with a three‐fold increase in the risk of death or refractoriness. Therefore, cTnI level should be considered as a prognostic indicator in patients diagnosed with TTP.
Abstract The prospective 1-year EPIC study enrolled 341 patients with myelodysplastic syndromes (MDS); although baseline iron burden was >2500 ng/mL, ∼50% were chelation-naïve. Overall median serum ...ferritin decreased significantly at 1 year ( p = 0.002). Decreases occurred irrespective of whether patients were chelation-naïve or previously chelated; changes were dependent on dose adjustments and ongoing iron intake. Sustained reductions in labile plasma iron were observed. Discontinuation rate (48.7%) and adverse event profile were consistent with previously reported deferasirox data in MDS. Alanine aminotransferase levels decreased significantly; change correlated significantly with reduction in serum ferritin ( p < 0.0001). This large dataset prospectively confirms the efficacy and well characterizes the safety profile of deferasirox in MDS.
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