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Case 123: Cardiac Hemosiderosis¹

¹ From the Department of Medical Imaging, University Health Network, Toronto General Hospital, 200 Elizabeth St, Toronto, ON, Canada M5G 2N2. Received May 11, 2004; revision requested July 28; revision received December 28; accepted January 21, 2005; final version accepted February 15.

Correspondence: Address correspondence to J.P. (e-mail: josephine.pressacco{at}icm-mhi.org).

	HISTORY

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
Computed tomography (CT) of the abdomen was performed to evaluate the liver in a 19-year-old woman who had undergone treatment for ß-thalassemia intermedia. CT revealed findings that prompted further investigation with cardiovascular magnetic resonance (MR) imaging. MR imaging was performed with a 1.5-T system (Signa Cvi; GE Medical Systems, Milwaukee, Wis). Selected CT and MR images are shown.

	IMAGING FINDINGS

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
CT of the abdomen revealed increased attenuation in the liver and spleen and normal attenuation in the pancreas (Fig 1). These findings indicated secondary hemochromatosis; iron overload in the liver and spleen and sparing of the pancreas are characteristic imaging findings of secondary hemochromatosis caused by reticuloendothelial deposition of iron in this patient with known thalassemia intermedia. Iron reduces the relaxation rates of T1- and T2-weighted sequences by introducing local magnetic field inhomogeneities (1,2). A coronal localizer image obtained with fast imaging employing steady-state acquisition (FIESTA; GE Medical Systems) (Fig 2) showed low signal intensity in the liver, spleen, and vertebral body marrow resulting from iron deposition.

View larger version (159K): [in this window] [in a new window] [Download PPT slide]   Figure 1: Selected unenhanced transverse CT scan of the abdomen (120 kV, 240 mA, 5-mm-thick section, 1.3 pitch, 512 x 512 matrix) demonstrates abnormally increased attenuation of the liver (L) and spleen (S) in contrast to the attenuation of the head of the pancreas (arrow), which is normal, and hyperattenuating lymph nodes immediately anterior to the inferior vena cava. These findings indicate iron overload in the reticuloendothelial system consistent with secondary hemochromatosis in a patient undergoing multiple red blood cell transfusions to treat thalassemia intermedia.

View larger version (186K): [in this window] [in a new window] [Download PPT slide]   Figure 2: Coronal fast imaging employing steady-state acquisition localizer MR image of the chest and upper abdomen obtained with a torso coil (repetition time msec/echo time msec, 760/20; 5-mm-thick section with 1-mm skip interval; 256 x 192 matrix; 32 x 32-cm displayed field of view). There is profound hypointensity of the liver (L), spleen (S), and vertebral bone marrow. These findings are consistent with a diagnosis of iron overload.

View larger version (126K): [in this window] [in a new window] [Download PPT slide]   Figure 3: Short-axis oblique T2-weighted MR image obtained through the left ventricular myocardium and the trapezius with a torso coil (3038/20, 7.0-mm section thickness, 3.0-mm spacing, 256 x 128 matrix). Normally, the myocardium demonstrates the same signal intensity as other skeletal muscles of the body. In this case, the myocardium demonstrates a substantial decrease in signal intensity compared with the skeletal muscle. This finding is in keeping with myocardial hemosiderosis in a patient with thalassemia treated with multiple red blood cell transfusions.

	DISCUSSION

TOP HISTORY IMAGING FINDINGS DISCUSSION References

Direct effects of thalassemia on organs and body tissues are secondary to profound anemia, by-products of hemolysis, and expansion of intra- and extramedullary erythroid marrow progenitors (4). Indirect effects are secondary to iron overload from accelerated iron turnover and are exacerbated by blood transfusions, leading to end-organ damage (5).

Patients present with microcytic anemia and hemoglobin levels as low as 3 g/dL. There is bone marrow erythroid hyperplasia with subsequent skeletal changes caused by expansion of erythroid bone marrow. A hair-on-end radiographic appearance of the skull is caused by widening of the diploic spaces and marrow expansion. Extramedullary hematopoiesis can occur and is a rare manifestation in other forms of chronic anemia (3–5).

Splenomegaly and hepatomegaly are common manifestations early in the disease and are caused by increased red blood cell destruction and extramedullary hematopoiesis in the spleen and liver. The kidneys are often enlarged secondary to the presence of extramedullary hematopoeisis. There is also a tendency for renal tubules to be dilated; this finding is not well understood. The high rate of cellular turnover causes hyperuricemia. Patients with thalassemia are at increased risk for developing gallstones and biliary tract inflammation from increased bilirubin production (3–5).

Cardiac abnormalities are a major concern in patients with thalassemia and include congestive heart failure, arrhythmia, and cardiac dilatation secondary to anemia (6). Anemia is corrected with blood transfusions. With each unit of transfused red blood cells, 200–250 mg of iron bypasses the gastrointesinal mucosal block; this excess iron is initially stored in macrophages. With continued transfusions, the excess iron spills over into hepatic parenchymal cells, myocardium, and endocrine cells and has the potential to cause tissue damage (7). Cardiac hemosiderosis is a feared complication in patients with thalassemia who have undergone a blood transfusion. An additional risk for damage in these patients seems to be the presence of the 4 allele of apolipoprotein E, which has decreased antioxidant and iron-binding activity (8,9). Sterile pericarditis, arrhythmias, and end-stage restrictive cardiomyopathy leading to congestive heart failure can develop in patients with cardiac hemosiderosis (10). Iron chelation therapy with deferoxamine can control the amount of iron overload; thus, it is considered a treatment for the complications caused by a red blood cell transfusion (11).

The differential diagnosis for the images presented here included many chronic anemias that can be treated with transfusions, such as sickle cell disease and other chronic hemolytic states, aplastic anemias, myelodysplastic syndromes, certain chronic leukemias, and pure red blood cell aplasia (especially Blackfan-Diamond syndrome). Hereditary iron overload unrelated to anemia, such as the rare African iron overload disease (12) or the common hereditary hemochromatosis (13), must be considered. Chronic alcohol abuse can also produce iron overload in body organs; however, there are often other stigmata of disease, including liver cirrhosis with vascular collateral vessels or varices. In the late stages of untreated myocardium iron overload, the appearance of the heart is similar to that seen with dilated cardiomyopathy. Knowledge of the patient's clinical history is needed to make the correct diagnosis. Thus, cardiac hemosiderosis was diagnosed in this patient who had a history of ß-thalassemia intermedial presentation.

	FOOTNOTES

 
Authors stated no financial relationship to disclose.

Part one of this case appeared 4 months previously and may contain larger images.

 

	References

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 

1. Mavrogeni SI, Gotsis ED, Markussis V, et al. T2 relaxation time study of iron overload in beta-thalassemia. MAGMA 1998;6(1):7–12. [Medline]
2. Westwood M, Anderson LJ, Firmin DN, et al. A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging 2003;18(1):33–39. [CrossRef][Medline]
3. Forget BG. Thalassemia syndromes. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: basic principles and practice. 3rd ed. New York, NY: Churchill Livingstone, 2000; 485.
4. Adams JG 3rd, Coleman MB. Structural hemoglobin variants that produce the phenotype of thalassemia. Semin Hematol 1990;27(3):229–238. [Medline]
5. Telfer PT, Prestcott E, Holden S, Walker M, Hoffbrand AV, Wonke B. Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major. Br J Haematol 2000;110(4):971–977. [CrossRef][Medline]
6. Kremastinos DT, Tsetsos GA, Tsiapras DP, Karavolias GK, Ladis VA, Kattamis CA. Heart failure in beta thalassemia: a 5-year follow-up study. Am J Med 2001;111(5):349–354. [CrossRef][Medline]
7. Hahalis G, Manolis AS, Gerasimidou I, et al. Right ventricular diastolic function in beta-thalassemia major: echocardiographic and clinical correlates. Am Heart J 2001;141(3):428–434. [CrossRef][Medline]
8. Ferrara M, Matarese SM, Francese M, et al. Role of apolipoprotein E (APOE) polymorphism on left cardiac failure in homozygous beta thalassaemic patients. Br J Haematol 2001;114(4):959–960. [Medline]
9. Miyata M, Smith JD. Apolipoprotein E allele-specific antioxidant activity and effects on cytotoxicity by oxidative insults and beta-amyloid peptides. Nat Genet 1996;14(1):55–61. [CrossRef][Medline]
10. Hahalis G, Manolis AS, Apostolopoulos D, Alexopoulos D, Vagenakis AG, Zoumbos NC. Right ventricular cardiomyopathy in beta-thalassaemia major. Eur Heart J 2002;23(2):147–156. [Abstract/Free Full Text]
11. Olivieri NF, Brittenham GM. Iron-chelating therapy and the treatment of thalassemia. Blood 1997;89(3):739–761. [Free Full Text]
12. Gordeuk VR, Caleffi A, Corradini E, et al. Iron overload in Africans and African-Americans and a common mutation in the SCL40A1 (ferroportin 1) gene. Blood Cells Mol Dis 2003;31(3):299–304. [CrossRef][Medline]
13. Beutler E, Hoffbrand AV, Cook JD. Iron deficiency and overload. Hematology Am Soc Hematol Educ Program 2003:40–61.

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