Systemic amyloidosis results from the deposition of insoluble, fibrous amyloid proteins. It occurs mainly in the extracellular spaces of multiple organs and tissues including the kidney, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinically apparent liver disease is relatively rare. Indeed, most patients with systemic amyloidosis manifest only minimal to moderate hepatomegaly and trivial abnormalities in liver function tests. Recently, we experienced two cases of patients who presented with abnormalities in liver function tests and hepatomegaly as manifestations of systemic amyloidosis. We report these cases with a review of the relevant literatures.
Adult ; Amyloidosis/complications/*pathology ; Female ; Hepatomegaly/complications/*diagnosis/pathology ; Humans ; *Liver Function Tests ; Male ; Middle Aged

We reported localized amyloidosis involving seminal vesicles and vasa deferentia, which was found in two patients with prostatic adenocarcinoma. A 60-yr-old (Case 1) and a 59-yr-old (Case 2) man came to our hospital with elevation of serum prostate-specific antigen (PSA) and biopsy proven carcinoma, respectively. MRI revealed multiple irregular foci of low signal intensity in the prostates as well as in both seminal vesicles and vasa deferentia on T2-weighted imaging, suggesting prostatic carcinoma with extension to both seminal vesicles and vasa deferentia in both cases. Under the clinical diagnosis of stage III prostatic adenocarcinoma, a radical prostatectomy was performed in both patients. Microscopically, Gleason score 7 adenocarcinoma was observed in both patients. In addition, isolated amyloidosis of both seminal vesicles and vasa deferentia was found without carcinoma involvement. Localized amyloidosis in the seminal vesicles, which is considered as senile process, has been occasionally reported in the autopsy and in the surgical specimens. Amyloid deposition in the vas deferens has also been reported in the literature, however, the deposition mimicking extension of carcinoma has not been reported. In this report, two cases of isolated amyloidosis of the seminal vesicles and vasa deferentia are described with electron microscopic study and literature review.
Adenocarcinoma/complications ; Amyloidosis/complications/*pathology ; Human ; Male ; Middle Aged ; Prostatic Neoplasms/complications ; Seminal Vesicles/*pathology ; Vas Deferens/*pathology

This paper describes the first case of common variable immunodeficiency (CVID) and AA amyloidosis. A recently treated tuberculosis, and chronic inflammation induced by frequent respiratory tract infections, were thought to be responsible for the amyloidosis. No other reason for this condition could be detected. Although T cell dysfunction in some CVID patients has been reported, pulmonary tuberculosis is quite rare with this condition. Bacterial or viral agents or evidence in favour of intestinal tuberculosis, which would explain this patient's recurrent diarrhea, were not found. In this case, the response of the attacks of diarrhea to metranidazole and the histologic observation of extensive intestinal amyloid deposition, which is known to decrease intestinal motility, made us conclude that the diarrhea was associated with bacterial overgrowth. In this report, we discuss the association of CVID and tuberculosis to secondary amyloidosis and recurrent diarrhea.
Tuberculosis/*complications ; Male ; Humans ; Diarrhea/etiology ; Common Variable Immunodeficiency/*complications ; Amyloidosis/*etiology/pathology ; Adult

Amyloidosis ; complications ; pathology ; Heart Diseases ; etiology ; pathology ; Humans ; Male ; Middle Aged

Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth. The symptoms of the disease are usually vague, while many kinds of auxiliary or laboratory examinations especially pathologic biopsy can provide a clue for the diagnosis. Here we described a case who had purpura-like lesions in the initial stage, followed by progressive malfunctions in the kidneys, the heart, the lungs, as well as the liver. The final diagnosis was primary systemic amyloidosis determined by skin pathologic biopsy. And the disease led to a fatal outcome within three months after the diagnosis.
Amyloidosis ; complications ; diagnosis ; diagnostic imaging ; Female ; Humans ; Middle Aged ; Purpura ; diagnostic imaging ; etiology ; pathology ; Ultrasonography

Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.
Female ; Humans ; Aged ; Glomerulonephritis/diagnosis* ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology* ; Antibodies, Antineutrophil Cytoplasmic ; Kidney/pathology* ; Amyloidosis/complications*

Aneurysms of the major thoracic veins are rare. They are usually asymptomatic and thus treated conservatively. We report an extremely rare case of rapidly progressing superior vena cava (SVC) aneurysm complicated by thrombosis and acute pulmonary thromboembolism (PTE) with right ventricular dysfunction. Thrombolytic therapy for hemodynamically significant acute PTE was harmful to the patient in the present case, because it induced further thrombosis and mobilization of the thrombi within the aneurysm, subsequently causing de novo PTE. Surgical aneurysmectomy combined with pulmonary artery embolectomy would be a treatment of choice in patients with SVC aneurysm complicated by acute PTE.
Abdominal Pain ; Amyloidosis/complications/*pathology ; Biopsy ; Diagnosis, Differential ; Endoscopy ; Humans ; Inflammatory Bowel Diseases/diagnosis ; Intestine, Small/*pathology ; Lymphoma, B-Cell, Marginal Zone/complications/*pathology ; Male ; Middle Aged

A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.
Abdominal Pain ; Amyloidosis/complications/*pathology ; Biopsy ; Diagnosis, Differential ; Endoscopy ; Humans ; Inflammatory Bowel Diseases/diagnosis ; Intestine, Small/*pathology ; Lymphoma, B-Cell, Marginal Zone/complications/*pathology ; Male ; Middle Aged

Tracheobronchopathia osteochondroplastica (TO) is a rare disorder of unknown cause characterized by the presence of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. A simultaneous diagnosis of TO and amyloidosis is rarely reported. In this report, a case initially suspected to be asthma bronchiole that could not be treated, was radiologically diagnosed as TO, and also secondary amyloidosis is presented. A 53 years, man patient reported a 3 years history of dyspnea. Pulmonary function tests (PFTs) showed an obstructive pattern. Chest X-rays revealed right middle lobe atelectasis. FOB and CT detected nodular lesions in the trachea and in the anterior and lateral walls of the main bronchi. AA amyloidosis was confirmed by endobronchial biopsy. In the abdominal fat pad biopsy, amyloidosis was not detected. Asthma bronchiole was excluded by PFTs. This case illustrates that it is possible for TO and amyloidosis to masquerade as asthma. TO and amyloidosis should be suspected in patients of older ages with asthma and especially with poorly treated asthmatic patients. Although nodular lesions in the anterior and lateral tracheobronchial walls are typical for TO, a biopsy should be obtained to exclude amyloidosis.
Amyloidosis/complications/*diagnosis/pathology ; Asthma/diagnosis ; Bronchial Diseases/complications/*diagnosis/radiography ; Diagnosis, Differential ; Humans ; Male ; Middle Aged ; Tracheal Diseases/complications/*diagnosis/radiography

In this report, we presented a case of solitary spine amyloidoma, its clinical and radiological findings and management, and a review of the literature on vertebral amyloidosis.
Amyloidosis/*complications/diagnosis/pathology ; Case Report ; Human ; Magnetic Resonance Imaging ; Male ; Middle Age ; Spinal Cord Compression/*etiology ; Spinal Diseases/*complications/diagnosis/pathology