11/380. Secondary hemochromatosis as a long-term complication of the treatment of hematologic malignancies. The increased cure rate of hematologic malignancies including the use of bone marrow transplantation has focused attention on the chronic toxicity and quality of life of the survivors. We have observed five patients who have been diagnosed with clinically significant iron overload, presumably due to packed red blood cell transfusions, >/=12 months after transplant for a hematologic malignancy. In these patients, there is no history of veno-occlusive disease or family history of hemochromatosis. The allotransplant patient has been free of chronic graft versus host disease. family screening has been negative. No patient developed clinically significant endocrinopathy, arthropathy, or cardiac disease. The patients have been treated with phlebotomy to bring the transferrin saturation and ferritin levels to normal. The long-term follow-up of patients treated for a hematologic malignancy should include analysis of hepatitis c virus and iron status. This may prevent the development of clinically significant chronic liver disease and possibly malignancy. ( info) |
12/380. Hepatocellular carcinoma in a patient with hereditary hemochromatosis and noncirrhotic liver. A case report. A case of a 62-year-old patient with hereditary hemochromatosis is reported, who developed hepatocellular carcinoma (HCC) in the absence of cirrhosis and other potential risk factors for HCC. Occurrence of HCC in patients with genetic hemochromatosis and noncirrhotic liver is a rare event which has previously been described only six times and appears to be limited to male patients. ( info) |
| BACKGROUND: hemochromatosis has been associated with atrial tachyarrhythmias and congestive heart failure as a consequence of dilated or restrictive cardiomyopathy. Inducible ventricular fibrillation has not been previously described.methods AND RESULTS: An electrophysiologic study was conducted in a woman after two episodes of syncope. Polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) were induced with ventricular programmed stimulation. magnetic resonance imaging demonstrated signal loss in the liver consistent with hemochromatosis, but normal cardiac size and function. Hematologic studies supported a diagnosis of hemochromatosis.CONCLUSION: Cardiac hemochromatosis may be associated with serious ventricular arrhythmias. ( info) |
14/380. hemochromatosis associated with endothelial dysfunction: evidence for the role of iron stores in early atherogenesis. Impaired endothelium-dependent, flow-mediated dilatation of the brachial artery was observed in a 50-year-old premenopausal female non-smoker with idiopathic hemochromatosis. Endothelial dysfunction observed in this patient supports a relationship between body iron stores and early atherosclerotic process. ( info) |
15/380. Hepatocellular carcinoma arising in the absence of cirrhosis in genetic haemochromatosis: three case reports and review of literature. Genetic haemochromatosis constitutes a high risk factor for the development of hepatocellular carcinoma. It is widely accepted that venesection prevents the evolution of cirrhosis in haemochromatosis and indirectly protects against the development of hepatocellular carcinoma. Clinical, pathological and radiological data are presented on three patients who did not conform to the 'siderosis-cirrhosis-carcinoma' sequence and in whom prompt and adequate iron depletion did not prevent the development of cancer. This is the first report of hepatocellular carcinoma intervening in non-cirrhotic liver in two siblings with genetic haemochromatosis. The current literature on the subject is reviewed. The direct oncogenic role of iron remains to be elucidated. Hepatocellular carcinoma should be considered as a differential diagnosis in patients with non-cirrhotic genetic haemochromatosis who present with clinical deterioration during the course of an otherwise uneventful venesection programme. ( info) |
16/380. Hereditary hemochromatosis: diagnosis and treatment in primary care. Hereditary hemochromatosis (HHC) is one of the most common inherited disorders in the Caucasian population. diagnosis usually made after an elevation in ferritin and serum transferrin saturation is noted, often accompanied by asymptomatic hepatomegaly. diagnosis is confirmed by genetic testing or liver biopsy. Damage to organs is due to excessive intestinal iron, which is transported to and then deposited in the liver parenchyma, and the heart, skin, and endocrine organs, causing skin pigmentation, development of cirrhosis and hepatic carcinoma, diabetes and endocrine failure, and heart failure. Bony changes can be manifested by arthritis, often in non-weight-bearing joints. The treatment of HHC is phlebotomy, which depletes iron stores. When diagnosis is made before organ damage occurs, treatment can prevent manifestations of the disease. skin pigmentation and some cardiac damage may reverse on depletion of iron stores, but liver and endocrine damage is rarely reversible. Arthropathy is also not reversible, and often continues to progress even with effective treatment. When hemochromatosis is diagnosed, all first degree relatives of the patient should undergo genetic testing. With early detection and treatment this can be a manageable chronic disease. If undetected, it is potentially fatal. ( info) |
17/380. diagnosis of neonatal hemochromatosis with MR imaging and duplex Doppler sonography. Neonatal hemochromatosis is a rare congenital disorder which affects both fetuses and newborns. It is characterized by hepatocellular failure, often appearing on the first day of life in the form of coagulopathy, hypoalbuminemia, hypoglycemia, and jaundice. Most of the affected infants die early in life, and definitive diagnosis has often been made only by post-mortem evaluation. With the help of MRI, plus increasing awareness of the disorder, diagnosis is now often made early, even in utero. Duplex Doppler sonography does not provide information on siderosis but shows abnormalities in the liver or blood-flow patterns associated with liver disease. ( info) |
18/380. Hepatocellular adenomatosis associated with hereditary haemochromatosis. A young healthy man presented with abdominal pain following an accidental fall. Imaging studies and laparoscopy revealed multiple yellowish well-defined hepatic lesions. Liver biopsies showed hepatic adenomas and iron overload. Laboratory investigation confirmed a diagnosis of hereditary haemochromatosis. To our knowledge this represents the first report of an association of hepatic adenomatosis and primary haemochromatosis. ( info) |
19/380. Hepatocellular carcinoma presenting as a pyogenic liver abscess in a patient with hemochromatosis. Primary hemochromatosis is rare in taiwan. Hepatocellular carcinoma (HCC) is endemic in taiwan, but HCC with initial presentation as pyogenic liver abscess is unusual. We report a case of HCC presenting as pyogenic liver abscess in a hemochromatotic patient with cirrhosis. The patient underwent hepatectomy and had a smooth postoperative course. Unfortunately, he died of pneumonia eight months after surgery. HCC should be considered in the differential diagnosis in hemochromatotic patients with a pyogenic liver abscess. ( info) |
20/380. cholangiocarcinoma arising in Von Meyenburg complex associated with hepatocellular carcinoma in genetic haemochromatosis. A 61-year-old man had a liver resection for a bilobar mass thought to be, by imaging techniques, an hepatocellular carcinoma. He had been treated for the last 12 years by venesections for genetic haemochromatosis complicated by well-compensated cirrhosis. At surgery, prothrombin time and platelet count were normal, as was alpha-fetoprotein. On the resected specimen, the non-tumoral liver was not cirrhotic; septal fibrosis was present as well as mild iron overload and numerous Von Meyenburg complexes. The bilobar tumour was composed of two different parts: one was a cholangiocarcinoma arising from Von Meyenburg complexes, the other was a moderately differentiated hepatocellular carcinoma with a partially invaded capsule. The two tumours, in close proximity, did not communicate. This observation raises three questions: the relative risk of primary liver cancer including both hepatocellular carcinoma and cholangiocarcinoma in haemochromatosis without cirrhosis; the development of cholangiocarcinoma from Von Meyenburg complexes; the reversibility of cirrhosis in treated patients. ( info) |