Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nodular regenerative hyperplasia of the liver was identified at autopsy in a patient with myelofibrosis with extramedulary hematopoiesis, an association not previously reported. Portal venous hypertension, documented during the patient's terminal hospitalization, was ascribed, in part, to a high rate of blood flow through the enlarged spleen. Possible mechanisms accounting for the development of nodular regenerative hyperplasia of the liver, and evidence provided by this case pertinent to these mechanisms, are discussed.
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PMID:Nodular regenerative hyperplasia of the liver in a case of myelofibrosis with extramedullary hematopoiesis and secondary portal venous hypertension. 45 99

Nodular regenerative hyperplasia (NRH) is a condition characterized by hepatocytic nodules distributed throughout the liver without perinodular fibrosis. The etiology is unknown, but it has been reported in patients with a variety of diseases, including autoimmune diseases and primary biliary cirrhosis (PBC). In this study, the liver biopsies of 64 patients with PBC were reviewed. Thirty-five biopsies in histological stages I or II belonging to 30 patients were suitable for study. NRH was found in 43% of biopsies (47% of patients). In 80% of these biopsies, nodular transformation was focal, while it was diffuse in the remaining 20%. Splenomegaly was more common in patients with NRH. Laparoscopy displayed evidence of portal hypertension in two of these patients and endoscopy showed esophageal varices in one patient. Serum levels of gamma-glutamyl transpeptidase were also higher in these patients. Lesions in small intrahepatic vessels, florid bile duct lesions, and portal granulomas were found more frequently in biopsies with NRH. We conclude that nodular hyperplastic changes are very common in early histological stages of PBC, and consequently, this disease should be considered in the etiology of NRH. These changes may contribute to early development of portal hypertension in these patients.
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PMID:Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis. 155 37

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon entity of unknown origin and pathogenesis. We report here a case of NRH of the liver which was associated with portal vein thrombosis and adrenal hyperplasia. A 48-year-old man who was admitted for further examination of portal hypertension and splenomegaly, died of acute myocardial infarction. Autopsy revealed an enlarged heart with occluded coronary arteries and fresh multiple necrosis in the myocardium. The spleen and the liver were enlarged. In the liver diffusely distributed nodules of regenerative hyperplasia were noted. Organized thrombi of the portal vein and adenomatous hyperplasia of the left adrenal gland were also noted. Antinuclear antibody was positive in the serum.
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PMID:Nodular regenerative hyperplasia of the liver with portal vein thrombosis and hyperplasia of the adrenal gland. 155 40

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.
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PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79

The authors review the symptomatology of splenic tumor pathologies in connection with 93 personal observations (71 non-Hodgkin lymphomas, 12 Hodgkin's disease, 6 metastases, 3 epidermoid cysts, 1 angioma). Nodular forms were observed in 31.2% of the cases reviewed. In most cases of lymphoma, the ultrasonographic finding was a homogeneous splenomegaly. The possibility of false negatives for splenic involvement in lymphomas when spleen volume is normal has led certain authors to suggest fine needle puncture of the spleen under ultrasonographic guidance. Such procedures are unnecessary if ultrasonography has detected an obvious lesion such as multinodular involvement of homogeneous splenomegaly. However, this moderately traumatic technique can provide valuable information until such time as ultrasonic tissue characterization has proven itself in vivo.
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PMID:[Echography in tumor pathology of the spleen: limitations and perspectives]. 666 34

Mastocytosis is a disease of mast cell hyperplasia that may involve several organ systems, including liver. Between 1988 and 1991, we conducted a retrospective-prospective study of 41 patients with mastocytosis and found 61% had evidence of liver disease. Hepatomegaly was detected in 24%, splenomegaly in 41%, and elevated serum alkaline phosphatase, serum aminotransaminases, 5'nucleotidase, or gamma-glutamyltranspeptidase (GGTP) in 54% of the patients. Alkaline phosphatase levels directly correlated with GGTP levels, hepatomegaly, splenomegaly, and liver mast cell infiltration and fibrosis. Elevated alkaline phosphatase levels and splenomegaly were observed more frequently in patients with categories II and III mastocytosis. Five patients in combined disease categories II or III developed ascites or portal hypertension and died of complications of mastocytosis; three had hypoprothrombinemia at the time of death. Thirty-five liver biopsy specimens from 25 patients were examined. Mast cell infiltration was commonly observed in the biopsy specimens, more severe in those patients with either category II or III disease, and correlated with hepatomegaly, splenomegaly, alkaline phosphatase levels, and GGTP levels. Mast cells were often only detected by using special stains (toluidine blue and chloracetate esterase). Increased portal fibrosis was seen in 68% of the biopsy specimens and correlated with mast cell infiltration and portal inflammation. Cirrhosis was not observed. Nodular regenerative hyperplasia, portal venopathy, and venoocclusive disease was observed in eight biopsy specimens and may have been the cause of the portal hypertension or ascites in four patients. These findings demonstrate that liver disease with mast cell infiltration is a common finding in patients with mastocytosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hepatic involvement in mastocytosis: clinicopathologic correlations in 41 cases. 755 67

The apparently high prevalence of splenomegaly in dogs, along with the surgical accessibility of the spleen, results in a relatively large number of splenectomies in dogs in clinical veterinary practice. Splenic nodular lesions are widely considered to be indicative of hemangiosarcoma and thus a disease that is ultimately fatal. This study correlates the results of complete pathologic evaluation and classification of 500 spleens obtained by splenectomy with survival information for each dog. Among the spleens examined, 257 of 500 (51.4%) were classified nonneoplastic and 241 (48.2%) were neoplastic; 2 (0.4%) were unclassified. Miscellaneous non-nodular splenomegaly accounted for 46 of 257 (18%) of the nonneoplastic lesions; nodular splenomegaly accounted for 206 of 257 (79%) of nonneoplastic splenic lesions and was composed of lymphoid hyperplastic nodules and associated hematomas, hyperplastic lymphoid nodules alone, or hematomas with no apparent underlying cause. Nodular neoplastic diseases of the spleen were divided among benign tumors (11.5%) and a variety of primary sarcomas. Hemangiosarcoma made up 51% of splenic malignancies but accounted for less than 25% of the spleens evaluated. Survival of dogs with hematomas associated with nonneoplastic conditions of the spleen was markedly different from that in dogs with hemangiosarcoma-associated hematomas, even though most could not be effectively differentiated on gross inspection. Two month postoperative survival was 83% for dogs with nonneoplastic-related hematomas, whereas only 31% of dogs with hemangiosarcoma, with or without associated hematomas, were alive after 2 months. Twelve-month survival times were 64% and 7%, respectively. An overall postsplenectomy survival rate of 52% was based on the number of dogs surviving for a minimum of 6 months postoperatively.
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PMID:Pathologic factors affecting postsplenectomy survival in dogs. 918 68

Nodular regenerative hyperplasia (NRH) of the liver is an infrequent entity that is usually diagnosed after the appearance of clinical signs of portal hypertension such as hepatomegaly, splenomegaly or upper gastrointestinal bleeding due to esophageal varices, which are the most frequently found clinical manifestations in NRH. Ascites is a less frequent finding and has always been described in association with other manifestations of portal hypertension. We describe a new case of NRH with atypical presentation in which ascites was the sole clinical manifestation.
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PMID:[Ascites as the sole clinical manifestation in a patient with nodular regenerative hyperplasia]. 1273 2

Nodular regenerative hyperplasia (NRH) of the liver is a rare disease that is characterized by multiple regenerative nodules in the hepatic parenchyma without fibrosis. The exact pathogenesis of NRH has not been established, but it's been suggested that obliteration of portal veins may initiate the nodular transformation. It is also known that this disease is associated with autoimmune disease, myeloproliferative disease, lymphoproliferative disease, primary biliary cirrhosis, and some chemotherapy agents. The patients with NRH are usually asymptomatic, yet if they have symptoms, the most common clinical manifestations are those of portal hypertension, including splenomegaly and esophageal varices with or without bleeding. We report a case of nodular regenerative hyperplasia that presented with clinical manifestations similar to those of primary biliary cirrhosis.
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PMID:[A case of nodular regenerative hyperplasia of liver that mimicked primary biliary cirrhosis]. 1561 7

Nodular regenerative hyperplasia of the liver (NRHL) is a very rare cause of portal hypertension and liver failure. The condition is characterized by diffuse micronodular transformation of hepatic parenchyma without fibrous septa between the nodules. We present our experience with a 32-year-old woman who presented with recurrent episodes of upper gastrointestinal bleeding associated with massive splenomegaly who was subsequently found to have NRHL. This article considers the salient aspects of this rare condition, how it affects the patients and the options available in its management. A plea is made for the need for liver biopsy for all patients with portal hypertension especially those being considered for surgery.
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PMID:Nodular regenerative hyperplasia of liver. 1841 67


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