scholarly journals Primary amyloidosis presenting intrahepatic cholestasis and fulminant hepatic failure

2015 ◽  
Vol 10 (1) ◽  
pp. 71
Author(s):  
Ozturk Ates ◽  
Kemal Kosemehmetoglu ◽  
Gunes Guner ◽  
Yusuf Bayraktar
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Intrahepatic Cholestasis ◽  
Monoclonal Gammopathy ◽  
Sinusoidal Obstruction Syndrome ◽  
Primary Amyloidosis ◽  
Hepatic Amyloidosis ◽  
Undetermined Significance

A 69-year-old man noticed abdominal pain located on right upper quadrant. Physical examination showed hepatosplenomegaly and icteric discoloration of sclera. On evaluation, patient was diagnosed to have hepatic amyloidosis related monoclonal gammopathy of undetermined significance (MGUS) and sinusoidal obstruction syndrome with intrahepatic cholestasis. In this case report we emphasize fulminant hepatic failure due to primer amyloidosis in diagnosed with MGUS patient.

scholarly journals Metastatic Breast Cancer Presenting as Fulminant Hepatic Failure: A Case Report and Literature Review

1989 ◽  
Vol 3 (4) ◽  
pp. 149-152 ◽  
Author(s):  
Malcolm S. Trimble ◽  
Cameron N. Ghent ◽  
David R. Grant ◽  
Caroline A. McLean
Keyword(s):  
Breast Cancer ◽  
Differential Diagnosis ◽  
Abdominal Pain ◽  
Metastatic Breast Cancer ◽  
Case Report ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Metastatic Breast ◽  
Metastatic Carcinoma ◽  
Metastatic Breast Carcinoma

A case of metastatic breast carcinoma presenting as fatal fulminant hepatic failure is presented. The patient did not have previously diagnosed breast cancer and presented with ascites, Jaundice, abdominal pain, nausea, vomiting and hepatic encephalopathy. She died within four weeks of the onset. Seven similar cases previously reported are reviewed. Metastatic carcinoma should he considered in the differential diagnosis of fulminant hepatic failure.

scholarly journals Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Ibrahim Altraif ◽  
Fayaz A. Handoo ◽  
Khaled O. Alsaad ◽  
Adel Gublan
Keyword(s):  
Alkaline Phosphatase ◽  
Case Report ◽  
Hepatic Failure ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Clinical Findings ◽  
Systemic Amyloidosis ◽  
Hepatic Involvement ◽  
Reactive Amyloidosis ◽  
Hepatic Amyloidosis

Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid.

Prognostic Factors for Malignant Transformation in Monoclonal Gammopathy of Undetermined Significance and Smoldering Multiple Myeloma

2002 ◽  
Vol 20 (6) ◽  
pp. 1625-1634 ◽  
Author(s):  
Clara Cesana ◽  
Catherine Klersy ◽  
Luciana Barbarano ◽  
Anna Maria Nosari ◽  
Monica Crugnola ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Extramedullary Plasmacytoma ◽  
Lymphocytic Leukemia ◽  
Cumulative Probability ◽  
Primary Amyloidosis ◽  
Smoldering Multiple Myeloma ◽  
Bence Jones Proteinuria ◽  
Undetermined Significance

PURPOSE: To evaluate the natural history of monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM), identify early predictors of evolution, and assess whether associated conditions correlate with disease progression. PATIENTS AND METHODS: A total of 1,231 consecutive patients with either MGUS (n = 1,104) or SMM (n = 127) diagnosed from July 1975 to March 1998 were included in the study. Cumulative survival probability and cumulative probability of transformation into lymphoproliferative disease were calculated by means of the Kaplan-Meier estimator. Univariate and multivariate Cox models were used to identify possible predictors of malignant evolution. RESULTS: Cumulative transformation probability at 10 and 15 years was 14% and 30%, respectively. At a median follow-up of 65 months (range, 12 to 239 months), 64 MGUS cases (5.8%) evolved to multiple myeloma (MM) (n = 43), extramedullary plasmacytoma (n = 1), primary amyloidosis (n = 1), Waldenström’s macroglobulinemia (n = 12), non-Hodgkin’s lymphoma (n = 6), and B-chronic lymphocytic leukemia (n = 1). At a median follow-up of 72 months (range, 12 to 247 months), 25 SMMs (19.7%) evolved to overt MM. A lower evolution risk was observed in MGUS than in SMM (P < .0001). Greater than 5% marrow plasmacytosis, detectable Bence Jones proteinuria, polyclonal serum immunoglobulin reduction, and high erythrocyte sedimentation rate (ESR) were independent factors influencing MGUS transformation. SMM progression correlated with greater than 10% marrow plasma cells, detectable Bence Jones proteinuria, and immunoglobulin (Ig) A isotype. Neither concomitant diseases nor immunosuppression correlated with progression. CONCLUSION: Careful evaluation of marrow plasmacytosis, urinary paraprotein, background immunoglobulins, ESR, and paraprotein isotype might help identify at presentation patients with benign monoclonal gammopathies requiring stricter monitoring.

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