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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred sixty-eight patients with primary systemic amyloidosis (AL) were identified. Median survival after diagnosis was 12 months and ranged from 4 months for patients presenting with congestive heart failure to 50 months for those presenting with peripheral neuropathy only. Utilizing the proportional-hazards model in a stepwise multivariate fashion to evaluate the simultaneous influence of putative risk factors as of diagnosis revealed that congestive heart failure, urine light chain, hepatomegaly, and multiple myeloma were the major factors adversely affecting survival during the first year after diagnosis. Serum creatinine, multiple myeloma, orthostatic hypotension, and monoclonal serum protein were the most important variables adversely affecting survival for patients surviving 1 year. These models were used to categorize patients according to the variables in the models into low-, moderate-, and high-risk groups for the first year after diagnosis and separately for subsequent years. The influence of these variables on survival is important in stratification of patients randomized to prospective clinical trials.
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PMID:Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases. 371 98

We describe two patients with primary systemic amyloidosis (AL) and the nephrotic syndrome. The administration of melphalan and prednisone was associated with resolution of the nephrotic syndrome. The serum albumin level returned to normal, proteinuria decreased to near normal, edema resolved, and the monoclonal protein in the serum and urine disappeared. In both patients, renal function remained stable and hepatomegaly disappeared. In both, however, amyloid deposition was greater in follow-up renal tissue than in the initial specimen. The effect of systemic therapy in AL must be assessed with histologic observations as well as clinical indexes.
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PMID:Primary systemic amyloidosis: resolution of the nephrotic syndrome with melphalan and prednisone. 710 24

Amyloidosis is a manifestation of a group of diseases resulting from the variable infiltration of multiple organs by a fibrillar protein called amyloid. Hepatic involvement in amyloidosis is common both in the primary and in the secondary forms, whereas clinically-dominant liver amyloidosis is relatively rare. The authors describe a case of primary systemic amyloidosis with giant hepatomegaly, portal hypertension and renal insufficiency; the patient did not develop jaundice, ascites or gastrointestinal bleeding but died 6 months later, death being due to cerebral haemorrhage.
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PMID:Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: a case report and a review of the literature. 828 79

We present the case of a 70-year-old woman who had been suffering from constitutional syndrome for several months, abdominal distension, and yellowish coloration of the skin for the previous few days with a rapidly fatal course. Examination revealed hepatomegaly and ascites. Laboratory investigations revealed hyperbilirubinemia with cholestasis. The remaining investigations (abdominal ultrasound, barium transit evaluation, bone marrow study, analysis of ascitic fluid and laparoscopy) did not establish the diagnosis. This was established by liver and subcutaneous fatty tissue biopsies, which revealed type AL amyloid deposits. Autopsy confirmed that the patient had primary systemic amyloidosis. This infrequent form of presentation of systemic amyloidosis and its poor prognosis are discussed.
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PMID:[Cholestatic jaundice and constitutional syndrome as early manifestations of primary systemic amyloidosis]. 1288 57

The liver is a common site of amyloid deposition in primary systemic amyloidosis. We reviewed the clinical features and natural history of patients with primary systemic amyloidosis and biopsy-proven hepatic involvement who were evaluated at Mayo Clinic from January 1, 1975, to December 31, 1997. The median age of the study group (68 men; 30 women) was 58.5 years. Seventy-one patients (72%) had involuntary weight loss. Hepatomegaly was found in 79 patients (81%). Eighty-two patients (89%) had proteinuria, and 81 patients (86%) had elevated serum alkaline phosphatase levels. Seventy-six patients (83%) had either a serum or urine monoclonal protein. Before liver biopsy, clinicians considered amyloidosis in the differential diagnosis for only 14 patients (26%). None of our patients experienced hepatic rupture or death due to liver biopsy, and only 4 (4%) bled after liver biopsy. The median survival of the 98 patients was 8.5 months. Predictors of a poor prognosis were congestive heart failure, elevated concentrations of bilirubin, and a platelet count greater than 500 x 109/L. In conclusion, clinicians should consider the diagnosis of primary hepatic amyloidosis in patients who present with involuntary weight loss or hepatomegaly. Other clues to the diagnosis include an unexplained elevated serum alkaline phosphatase level, proteinuria, and evidence for hyposplenism (for example, Howell-Jolly bodies on peripheral blood smear). Liver biopsy was safe. Some patients benefit from systemic chemotherapy.
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PMID:Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. 1453 Jul 78

Although the liver and spleen are frequently involved in primary systemic amyloidosis, the clinical manifestations of hepatic and splenic involvement are usually mild and a dominant symptomatic hepatic presentation is uncommon. We report a case of a 51-year-old woman with giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, and ascites, in whom the findings of dual-phase spiral computed tomography suggested liver and splenic amyloidosis.
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PMID:Hepatic and splenic amyloidosis: dual-phase spiral CT findings. 1462 77

Primary systemic amyloidosis is a rare disease with protean manifestations. Presence of nephrotic syndrome in the absence of diabetes and hypertension, cardiomyopathy in the absence of ischemia, restrictive cardiac defect, demyelinating polyneuropathy, or unexplained hepatomegaly should alert the physician to the possibility of amyloidosis. Initial steps in the diagnostic evaluation of patients with suspected amyloidosis include serum and urine immunoelectrophoresis and immunofixation studies. Demonstration of amyloid material on tissue biopsy (e.g., subcutaneous fat) is required for diagnosis. Availability of effective treatments has improved the outlook of patients with primary systemic amyloidosis. Early diagnosis is critical to optimizing the chances of effective therapy.
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PMID:Primary systemic amyloidosis. Early diagnosis and therapy can improve survival rates and quality of life. 1691 52

The liver is a common site of amyloid deposition in primary systemic amyloidosis. We report the case of a 52-year-old white woman complaining of hepatomegaly, high levels of alkaline phosphatase and serum gamma-glutamyl transferase. Other laboratory tests showed proteinuria with light-chain type lambda. Color Doppler ultrasonography showed an enlarged bright liver with hepatopetal portal blood flow. Fine-needle aspiration biopsy of abdominal fat, with Congo red stain, was positive for amyloid. No liver biopsy was performed, but transient elastography showed high liver stiffness values (75 kPa), suggestive of amyloid infiltration, as other causes of elevation had been ruled out by clinical, laboratory and radiological findings. Bone marrow morphology and immunoistochemistry confirmed low-grade plasmacytoma with amyloidosis.
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PMID:Liver AL amyloidosis as a possible cause of high liver stiffness values. 2104 65

A 65-year-old male presented with hemorrhagic bullous skin lesions with purpura and ecchymoses. There was increased skin fragility with a strongly positive Nikolsky sign. Histopathology of the skin revealed large amounts of amyloid deposits in the dermis with a positive Congo Red staining around the dermal vessels. Examination and tests in this patient also revealed anemia, hepatomegaly, infiltrative cardiomyopathy, polyneuropathy and immunoglobulin l deposition, favoring a diagnosis of primary amyloidosis (AL type). The present case is reported in view of the rarity of the bullous variant of primary systemic amyloidosis as well as presence of mucosal lesions and a positive Nikolsky sign.
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PMID:Systemic amyloidosis presenting as mucocutaneous bullous lesions. 2013 56