Hepatic and cardiac iron load as determined by MRI T2* in patients with congenital dyserythropoietic anemia type I
- PDF / 498,831 Bytes
- 6 Pages / 595.276 x 790.866 pts Page_size
- 76 Downloads / 162 Views
ORIGINAL ARTICLE
Hepatic and cardiac iron load as determined by MRI T2* in patients with congenital dyserythropoietic anemia type I Mahdi Asleh 1
&
Aviva Levitas 2 & Sharon Daniel 3 & Abed Abu-Quider 1 & Miriam Ben-Harosh 1 & Joseph Kapelushnik 1
Received: 27 July 2020 / Accepted: 7 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Iron overload comprises one of the main complications of congenital dyserythropoietic anemia type I (CDA-I). When analyzing magnetic resonance imaging T2* (MRI T2*) results in CDA patients, two previous studies reported discordant results regarding iron load in these patients. To further understand iron loading pattern in this group of patients, we analyzed MRI T2* findings in 46 CDA-I patients. Mild to moderate hepatic iron overload was detected in 28/46 (60.8%) patients. A significant correlation was found between serum ferritin and liver iron concentration (LIC). A significant correlation (p value = 0.02) was also found between the patient’s age and LIC, reflecting increased iron loading over time, even in the absence of transfusion therapy. Notably, no cardiac iron overload was detected in any patient. Transfusion-naive patients had better LIC and better cardiac T2* values. These results demonstrate that a high percentage of CDA-I patients have liver iron concentration above the normal values, risking them with significant morbidity and mortality, and emphasize the importance of periodic MRI T2* studies for direct assessment of tissue iron concentration in these patients, taking age and transfusional burden into consideration. Keywords Congenitaldyserythropoietic anemia . Ironoverload . Magneticresonance imaging . Ineffective erythropoiesis . Serum ferritin
Introduction Congenital dyserythropoietic anemia type I (CDA-I) is one of a heterogeneous group of disorders of erythropoiesis. It is characterized by ineffective erythropoiesis as the predominant cause of anemia and by distinct morphologic abnormalities of erythroblasts in bone marrow including binucleate macrocytic erythroblasts and internuclear bridging [1]. CDA-I may present in infancy or adulthood and is characterized by moderate-
* Mahdi Asleh [email protected] 1
Pediatric Hemato-oncology department, Saban Pediatric Medical Center, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Rager St. 151, Beer-Sheva, Israel
2
Pediatric Cardiology Unit, Saban Pediatric Medical Center, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel
3
Clalit Health Services, Soroka University Medical Center, Department of Pediatrics and Public health, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel
to-severe macrocytic anemia, usually accompanied by jaundice and splenomegaly [2]. While only a small percentage of patients are transfusion dependent, increased iron burden is universal, and iron overload comprises one of the main complications of the dis
Data Loading...
