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*

A 53-year-old woman was admitted with epigastric discomfort and weakness. Laboratory examination at admission showed mild anemia and proteinuria. Esophagogastroduodenoscopy revealed marked mucosal atrophy, diffuse nodularity and granular appearance with mucosal friability. Biopsy was performed on the antrum and body of the stomach. On the next day, the patient began to complain of severe dyspnea, and hypoxia was present on pulse oximetry. Therefore, emergency echocardiography was conducted and it showed restrictive cardiomyopathy along with thrombus in the left atrium. With time, heart failure was aggravated despite intensive management. The result of gastric biopsy revealed amyloid deposits which stained positively with Congo red. On immunohistochemistry study, kappa and lambda chain were present. In addition, kappa chain was significantly elevated in urine and serum on electrophoresis. Although the patient was finally diagnosed as having primary gastric amyloidosis with restrictive cardiomyopathy, her general condition rapidly deteriorated and died at 12th hospital day. When obscure gastric lesion is encountered, performing gastric biopsy is strongly recommended since it be primary gastric amyloidosis. Herein, we present an unusual case of primary gastric amyloidosis.
Amyloidosis/complications/*diagnosis/pathology ; Endoscopy, Digestive System ; Female ; Heart Atria/diagnostic imaging ; Heart Failure/complications/*diagnosis ; Humans ; Immunoglobulin kappa-Chains/blood/urine ; Immunoglobulin lambda-Chains/blood/urine ; Immunohistochemistry ; Magnetic Resonance Imaging ; Middle Aged ; Stomach Diseases/complications/*diagnosis/pathology ; Thrombosis/diagnosis/diagnostic imaging ; Tomography, X-Ray Computed ; Ultrasonography

No abstract available.
Amyloidosis/complications/*diagnosis/pathology ; Colitis, Ulcerative/diagnosis ; Colonoscopy ; Gastrointestinal Hemorrhage/*etiology ; Heart Ventricles/radiography ; Humans ; Magnetic Resonance Imaging ; 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

We report herein a case of intestinal amyloidosis with grave prognosis that caused intractable diarrhea and intestinal pseudo-obstruction, alternately in spite of intensive conservative treatment. A 44-year-old woman was admitted for fever, diarrhea, and crampy abdominal pain which had been continuned during 6 months. Abdomen CT scan showed edematous wall thickening of the small bowel and right colon, and colonoscopic biopsy revealed amyloid deposition in the mucosa. Monoclonal light chains in serum and/or urine were not detected and highly elevated serum amyloid A was shown. In spite of intensive treatment including oral prednisolone and colchicine, diarrhea and intestinal pseudo-obstruction developed alternately, general status rapidly got worsened and died after two months.
Administration, Oral ; Adult ; Amyloidosis/complications/*diagnosis/drug therapy ; Anti-Inflammatory Agents/therapeutic use ; Colchicine/therapeutic use ; Colonoscopy ; Diarrhea/*etiology ; Female ; Humans ; Intestinal Mucosa/pathology ; Intestinal Pseudo-Obstruction/*diagnosis/etiology ; Prednisolone/therapeutic use ; Serum Amyloid A Protein/metabolism ; Tomography, X-Ray Computed ; Tubulin Modulators/therapeutic use

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

We report here on a rare case of primary AL hepatic amyloidosis associated with multiple myeloma in a 64-year-old woman. The patient was referred for evaluating her progressive jaundice and right upper quadrant pain. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) showed diffusely and markedly increased 18F-FDG uptake in the liver. Although there have been several case studies showing positive 18F-FDG uptake in pulmonary amyloidosis, to the best of our knowledge, the 18F-FDG PET/CT findings of hepatic amyloidosis or primary hepatic amyloidosis associated with multiple myeloma have not been reported previously.
Amyloidosis/complications/pathology/*radionuclide imaging ; Biopsy, Needle ; Female ; Fluorodeoxyglucose F18/*diagnostic use ; Humans ; Liver/pathology ; Liver Diseases/complications/pathology/*radionuclide imaging ; Middle Aged ; Multiple Myeloma/*complications ; *Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/*diagnostic use

BACKGROUND AND OBJECTIVEAmyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.

METHODSClinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR).

RESULTSCase 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases.

CONCLUSIONSOur findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

Aged ; Amyloidosis ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Antigens, CD ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Elbow ; Follow-Up Studies ; Head and Neck Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Interferon Regulatory Factors ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Skin Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

Amyloid ; metabolism ; Amyloidosis ; complications ; diagnosis ; pathology ; Biopsy, Fine-Needle ; Congo Red ; Female ; Hepatomegaly ; etiology ; Humans ; Liver ; metabolism ; pathology ; Liver Diseases ; diagnosis ; etiology ; pathology ; Liver Function Tests ; Middle Aged ; Tomography, X-Ray Computed