Cases reported "Liver Diseases"

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1/85. Reactive lymphoid hyperplasia of liver coexisting with chronic thyroiditis: radiographical characteristics of the disorder.

    BACKGROUND: Reactive lymphoid hyperplasia of the liver is an extremely rare entity, with six cases reported so far. methods: We encountered a 47-year-old Japanese female with reactive lymphoid hyperplasia of the liver, which coexisted with chronic thyroiditis. The lesion was discovered incidentally as a hypo-echoic mass with a hyper-echoic rim at a routine ultrasonography examination. It increased from 12 to 17 mm diameter in 6 months. Radiological studies, such as contrast-enhanced computerized tomography (CT) and angiography demonstrated a hypervascular lesion. RESULTS: It was consequently diagnosed as a neoplasm with malignant potentiality and she underwent partial hepatectomy. The lesion was composed of small mature lymphocytes which formed prominent lymphoid follicles with germinal centres, scattered plasma cells and stromal fibrosis. Immunohistochemical study revealed polyclonal origins of the involved lymphocytes. dna analysis for the immunoglobulin heavy gene and the T cell receptor beta gene using Southern blot hybridization showed no monoclonality. The following features have characterized the images in past cases, as well as ours: hypo-echoic mass, occasionally with a rim, in ultrasonography and hypervascularity, shown by angiography and enhanced CT.
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2/85. Nodular lymphoid lesion of the liver: an immune-mediated disorder mimicking low-grade malignant lymphoma.

    Three cases of unusual lymphoid infiltrate forming nodular macroscopic masses in the liver were studied in the authors' surgical pathology laboratory. These lesions posed difficulty in diagnosis, and their differentiation from low-grade lymphoma was not possible on histopathologic evaluation alone. The liver masses were analyzed histologically and immunohistochemically as well as for clonal immunoglobulin heavy chain (IgH) and T-cell receptor gamma (TCR-gamma) gene rearrangements. The lesions were seen as solitary grossly distinct firm nodules in all three patients, measuring 0.4, 0.7, and 1.5 cm, respectively, in their greatest dimensions. Two were found in livers removed because of end-stage primary biliary cirrhosis at the time of orthotopic liver transplantation, and the third was an incidental finding during laparotomy. Microscopically, these were nodules composed of small lymphocytes, plasma cells, and immunoblasts, with varying degrees of admixed acute inflammatory cells and scattered lymphoid follicles. By immunohistochemistry and molecular studies, these were found to be reactive lymphoid proliferations. All patients are alive and well at 2, 4, and 13 years, respectively. It is concluded that these cases represent a unique type of nodular lymphoid lesion, which is probably an immune-mediated benign reactive hyperplasia. It constitutes an entity by itself and must be distinguished from low-grade lymphoma. For a definitive diagnosis, immunohistochemistry and molecular studies are required.
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3/85. Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature.

    Nodular regenerative hyperplasia of the liver (NRHL) is a rare disorder characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the nodules. This condition appears to be associated in many occasions with systemic autoimmune diseases. We describe two new patients with NRHL in whom antiphospholipid antibodies (aPL) were detected in their sera and review the few similar cases reported previously. We also discuss the possible relationship between aPL and NRHL and suggest that these antibodies may play a role in the pathogenesis of some cases of NRHL, specially those with an associated antiphospholipid syndrome.
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4/85. Imaging features of nodular regenerative hyperplasia of the liver mimicking hepatic metastases.

    We described the sonographic, computed tomographic (CT), and magnetic resonance (MR) imaging features of one atypical case of nodular regenerative hyperplasia of the liver. The presence of multiple hepatic nodules suggested the diagnosis of metastatic disease to the liver because of a peripheral rim of enhancement on CT obtained after intravenous administration of contrast material and a halo sign on T2-weighted spin-echo MR imaging. Examination of the pathologic specimen obtained after surgical biopsy showed that the nodules were made of hepatocytes, with a nodular arrangement surrounded by peliosis, without fibrosis or cirrhosis. These findings suggested that peliosis may cause peripheral rim of enhancement on CT and halo sign on MR imaging. In light of this case, nodular regenerative hyperplasia of the liver should be considered in the differential diagnosis of hepatic metastases.
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5/85. A case of Schnitzler's syndrome with nodular regenerative hyperplasia of the liver.

    Schnitzler's syndrome is a rare condition of urticaria, macroglobulinemia, and sclerotic bone lesions. We report a case in a 70-year-old man in whom inflammatory polyarthralgia was followed by a nonpruritic urticarial eruption with a moderate decline in general health. Laboratory tests showed inflammation and a modest isolated peak of monoclonal IgM kappa. There was no evidence of waldenstrom macroglobulinemia. Schnitzler's syndrome was considered. However, an ultrasound scan of the abdomen done because of mild gamma-glutamyl-transferase elevation disclosed multiple hepatic lesions. The liver histology showed incipient nodular regenerative hyperplasia. Only about 30 cases of Schnitzler's syndrome have been reported since the seminal description in 1972. Hepatic involvement was a common but nonspecific finding, and we found no cases with nodular regenerative hyperplasia. However, this abnormality is often found in patients with autoimmune or hematological disorders. The pathogenesis of Schnitzler's syndrome remains unknown, but the possibility of progression to a hematological malignancy requires prolonged follow-up.
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6/85. Hepatic manifestations of familial patent ductus venosus in adults.

    BACKGROUND: The ductus venosus connects the umbilical vein to the inferior vena cava during fetal life and subsequently closes rapidly after birth. It is known as patent ductus venosus when it remains patent in adulthood. patients: A 43 year old man with a history of panhypopituitarism presented with recurrent bouts of pedal oedema associated with fatigue, hypoalbuminaemia, and elevated prothrombin time. An ultrasound examination of his abdomen with Doppler revealed notable attenuation of the main portal vein with diminished intrahepatic branches; a computed tomography scan with angiography revealed a large collateral vein within the liver consistent with a patent ductus venosus. Sequential liver biopsies showed a considerable reduction in the calibre and number of the portal veins. His younger brother, who was diagnosed with alcohol related cirrhosis, suffered from intermittent bouts of encephalopathy and was found to have the same vascular lesion. A third brother was found to have a patent ductus venosus as well as two large hepatic masses consistent with focal nodular hyperplasia. CONCLUSION: The syndrome of familial patent ductus venosus has only previously been described in three infant brothers who presented with hepatic encephalopathy and fatty degeneration of the liver. This report documents three brothers with a patent ductus venosus presenting in adulthood with different manifestations of liver disease. The presence of the same vascular anomaly in three brothers is highly suggestive of a recessive genetic trait with an anatomical manifestation of patent ductus venosus.
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7/85. Marked histiocytosis in the portal tract in a patient with reactive hemophagocytic syndrome: An autopsy case.

    We report an autopsy case of reactive hemophagocytic syndrome with peculiar liver histology. A 71-year-old female was diagnosed as having acute myelogenous leukemia and treated with chemotherapy. During her course, methicillin-resistant staphylococcus aureus (MRSA) was noted in blood culture and she was diagnosed as having MRSA sepsis. She died of respiratory failure 5 months after the onset of leukemia and 10 days after the MRSA sepsis. Ante-mortem liver function tests were within normal ranges. At autopsy, myeloblastic leukemia cells positive for CD13 were present in the bone marrow and, to a much lesser extent, in the spleen and liver. Numerous histiocytes of a bland appearance with erythrophagocytosis were noted in the bone marrow and spleen. The histiocytes were positive for CD68, but negative for S-100 and lysozymes. In the liver, many histiocytes of bland appearance with erythrophagocytosis and CD68 positivity were present in the portal tracts with no Kupffer cell hyperplasia. There were no hepatocellular degeneration, fatty changes or sinusoidal dilations. We consider that this histiocytosis was associated with MRSA infection and diagnosed this as infection-associated hemophagocytic syndrome. In previously reported cases, hemophagocytosis in hyperplastic kupffer cells was the main liver change of reactive hemophagocytic syndrome. The present case suggests that marked histiocytosis in portal tracts only may be a main feature of liver changes in reactive hemophagocytic syndrome and that such cases may not show abnormal liver function tests.
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8/85. Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature.

    BACKGROUND: Nodular regenerative hyperplasia (NRH) of the liver is a multi-acinar regenerative nodular lesion in a non-cirrhotic liver. It is a rare entity, especially in children, and remains of unknown aetiology. OBJECTIVE: NRH is often seen in association with other diseases or drug intake. In half of patients it is complicated by portal hypertension. Radiologically, its nodular appearance may look like neoplasia. RESULTS: We report a case of NRH with enormous hepatomegaly and multiple huge nodules. CONCLUSION: We wish to emphasise the importance of open wedge biopsy to establish diagnosis, since the prognosis of NRH in the absence of portal hypertension is good. Complications such as rupture of a nodule are rare.
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9/85. Hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta.

    A rare case of prenatally diagnosed hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta is presented and the literature reviewed. The placenta was noticed to have multiple cysts at 16 weeks' gestation and elevated maternal serum alpha-fetoprotein was present. Cystic liver masses were first detected in the fetus at 34 weeks' gestation. The liver mass showed a progressive enlargement during the third trimester and after birth, necessitating extended left hepatic trisegmentectomy at 3 months of age. Histological examination established the diagnosis of mesenchymal hamartoma of the liver and mesenchymal stem villous hyperplasia of the placenta. This is the first report of this association in which both lesions were diagnosed in utero and confirmed by histology after delivery. The likely pathogenesis of these strikingly similar lesions is discussed.
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10/85. Pseudotumorous hyperplasia of the caudate lobe in the non-cirrhotic liver: MR and CT arterial portography appearance.

    We describe the magnetic resonance image and computed tomographic arterial portography appearance of pseudotumorous hyperplasia in the caudate process of the caudate lobe in a non-cirrhotic patient. A prominent portal vein branch directly arising from the right main portal branch was seen in the center of the lesion on magnetic resonance imaging and computed tomographic arterial portography. We think that portal flow change of the caudate lobe by an anomalous portal branch may be correlated to pseudotumorous hyperplasia.
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