Aged ; Amyloidosis ; complications ; Factor X Deficiency ; complications ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male

Aged ; Amyloidosis ; complications ; diagnosis ; Diagnostic Errors ; Dyspnea ; etiology ; Edema ; etiology ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis

Abdominal Pain/etiology* ; Amyloidosis/diagnosis* ; Humans ; Immunoglobulin Light-chain Amyloidosis/complications* ; Immunoglobulin lambda-Chains

Adult ; Amyloidosis ; complications ; Factor X Deficiency ; complications ; Humans ; Liver Diseases ; complications ; Male

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

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 is a disorder characterized by extracellular deposition of amyloid in various tissues and organs. Gastrointestinal manifestations including gastroparesis, constipation, malabsorption, intestinal pseudo-obstruction, and bleeding are common. GI bleeding is a rare initial symptom which can be fatal in some cases. Absence of systemic symptoms and nonspecific endoscopic findings in amyloidosis may make diagnosis difficult. Therefore, amyloidosis-induced GI bleeding should be considered in patients with an obscure hemorrhage. Recently, we experienced a 65-year-old woman who presented with massive hematochezia as a manifestations of amyloidosis. Colonoscopy and SMA angiography showed massive bleeding in the small and large intestine. Colonoscopic biopsy established amyloidosis. We report this case with a review of the relevant literatures.
Aged ; Amyloidosis/*complications/diagnosis ; Female ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Intestinal Diseases/*complications/diagnosis

The study was aimed to investigate the effective therapeutic method for patients with multiple myeloma accompanied with amyloidosis. A 58-year-old patient diagnosed as multiple myeloma accompanied with amyloidosis in four limbs was enrolled in this study. The various clinical and laboratorial examinations were performed, including bone marrow smear, immunologic test, radiography and so on. Patient received chemotherapeutic drugs and then autologous hematopoietic stem cell transplantation (auto-HSCT). The result showed that hematopoietic reconstitution was achieved at 23 days after auto-HSCT. Immunofixation electrophoresis was normal. There was only 0.6% plasma cells in the bone marrow. In conclusion, the auto-HSCT may be an effective treatment for multiple myeloma accompanied with amyloidosis in four limbs.
Amyloidosis ; complications ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Middle Aged ; Multiple Myeloma ; complications ; surgery ; Transplantation, Autologous

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

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