Journal
AMERICAN JOURNAL OF HEMATOLOGY
Volume 89, Issue 7, Pages 678-683Publisher
WILEY
DOI: 10.1002/ajh.23721
Keywords
-
Categories
Funding
- Shire
Ask authors/readers for more resources
Chronically transfused sickle cell disease (SCD) patients have lower risk of myocardial iron overload (MIO) than comparably transfused thalassemia major (TM) patients. However, cardioprotection is incomplete. We present the clinical characteristics of six patients who have prospectively developed MIO, to identify potential risk factors for cardiac iron accumulation. From 2002 to 2011, cardiac, hepatic, and pancreatic iron overload were assessed by R-2 and R-2* magnetic resonance imaging techniques in 201 chronic transfused SCD patients as part of their clinical care. At the time, they developed MIO, five of six patients had been on chronic transfusion for more than 11 years; only one was on exchange transfusion. The time to MIO was correlated with reticulocyte and hemoglobin S percentages. All patients had qualitatively poor chelation compliance (<50%). All patients had serum ferritin levels >4600 ng/ml and liver iron concentration >22 mg/g. Pancreatic R-2* was >100 Hz in every patient studied (5/6). Cardiac iron rose proportionally to pancreas R-2*, with all patients having pancreas R-2*>100 Hz when cardiac iron was present. MIO had a threshold relationship with liver iron that was higher than observed in TM patients. In conclusion, MIO occurs in a small percentage of chronically transfused SCD patients and is only associated with exceptionally poor control of total body iron stores. Duration of chronic transfusion is clearly important but other factors, such as levels of effective erythropoiesis, appear to contribute to cardiac risk. Pancreas R-2* can serve as a valuable screening tool for cardiac iron in SCD patients. (C) 2014 Wiley Periodicals, Inc.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available