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PubMed2000

[A case of localized nodular pulmonary amyloidosis diagnosed by video-assisted thoracoscopic surgery].

Pulmonary amyloidosis is a rare pulmonary lesion. We report a 42-year-old male admitted for further evaluation of abnormal chest shadow. A chest CT demonstrated a nodular shadow in right S3. We tried transbronchial and percutaneous lung biopsy, but could not diagnose. We performed partial lung resection by video-assisted thoracoscopic surgery. Pathological diagnosis of the lesion was pulmonary amyloidosis. Immunohistochemical examination revealed this amyloid composed of AL kappa protein. No clinical evidence of amyloid deposits were found in other organs. We finally diagnosed this case as a localized nodular pulmonary amyloidosis.

Adult↗
PubMed2000

[Pericardial effusion revealing cardiac amyloidosis in the course of rheumatoid arthritis].

Pericardial effusion is common in patients with rheumatoid arthritis. It is essentially a sign of pericardial involvement of the rheumatoid disease, but viral, bacterial and especially tuberculous pericarditis must not be excluded. Pericardial amyloidosis of the AA type is much less common and difficult to diagnose before cardiac biopsy even in cases of myocardial amyloidosis, as in the reported case, in which the classical association of microvoltage on the ECG and myocardial hypertrophy on echocardiography was absent. The absence of myocardial uptake of technetium-labelled pyrophosphates at myocardial scintigraphy and the absence of a restrictive profile on cardiac gamma-angiography were not suggestive of the diagnosis of amyloidosis. Pericardial and endomyocardial biopsy, justified by the negativity of the preceding investigations, provided an accurate histological diagnosis, a prognostic evaluation and was also useful for guiding management.

Aged↗
PubMedDate not supplied

Successful treatment of protein-losing enteropathy due to AA amyloidosis with somatostatin analogue and high dose steroid in ankylosing spondylitis.

Secondary amyloidosis is an occasional complication of ankylosing spondylitis (AS) and in most cases renal amyloidosis presents with proteinuria, nephrotic syndrome and decreased renal function. We describe a 32-year-old male patient with AS manifested by frequent diarrhea, intermittent abdominal pain and low serum albumin levels. He has suffered from severe inflammatory back pain for 14 years with multiple peripheral joint involvement. Protein-losing enteropathy due to gastrointestinal amyloidosis was diagnosed with 99mTc-human albumin scintigraphy, fecal alpha-1 antitrypsin clearance and colonoscopic biopsy with Congo red staining. Somatostatin analogue octreotide and prednisolone were introduced with successful result.

Adult↗
PubMed2000

Giant cell arteritis "causing" AA-amyloidosis with rapid renal failure.

Giant cell arteritis (GCA) is a systemic vasculitic disease, which in very rare cases causes inflammatory complications such as secondary amyloidosis. We describe a well-documented case, with a clinically mild course, of biopsyproven giant cell arteritis as the only apparent cause of systemic AA-Amyloidosis. The deterioration in renal function due to amyloid deposition occurred rapidly and only a few months after manifestation of giant cell arteritis and was not reversible by steroid treatment. The renal arteries were normal and there was no glomerulonephritis due to giant cell arteritis. This unique case demonstrates that giant cell arteritis with a mild clinical course is closely associated with early-onset severe secondary amyloidosis.

Acute Kidney Injury↗
PubMed2000

Localised nodular pulmonary amyloidosis in a patient with sicca syndrome.

A 52 year old Chinese woman with a 25 year history of sicca syndrome (primary Sjogrens syndrome) was investigated for 3 episodes of haemoptysis. Clinical examination was unremarkable except for the presence of dry eyes and xerostomia. Computed tomography of the chest revealed a lobulated mass in the posterior basal segment of the left lower lobe. Histopathological examination of this resected nodule confirmed the diagnosis of nodular amyloidosis. The normal radiolabelled serum amyloid P component scintigraphy and the absence of monoclonal plasma cell dyscrasia in the bone marrow strongly support the diagnosis of localised nodular pulmonary AL amyloidosis in this patient. Nodular pulmonary amyloidosis can be associated with sicca syndrome and often simulates bronchogenic carcinoma, bronchiectasis or pulmonary tuberculosis.

Amyloidosis↗
PubMed2001

[Adrenocortical insufficiency in renal amyloidosis].

Renal involvement in amyloidosis leads to chronic renal failure. Prognosis is poor. Although amyloid deposits are frequent in adrenal glands, symptomatic adrenal dysfunction is uncommon. We report the case of a 63-year-old man with chronic renal failure (serum creatinine: 202 micromol/L) subsequent to amyloidosis who was referred to our unit for vomiting, dehydration despite a persistent nephrotic syndrome, acidosis, hyponatremia (121 mmol/l) and hyperkaliemia (7.1 mmol/l). A synacthen test was performed and disclosed adrenal insufficiency. Despite the initiation of substitution therapy, the patient died one month later from Addisonian crisis. Features of adrenal insufficiency may be masked by those of chronic renal failure, emphasizing the importance of adrenal explorations in patients with chronic renal failure due to amyloidosis.

Adrenal Insufficiency↗
PubMed2001

Successful treatment of endobronchial amyloidosis using Nd:YAG laser therapy as an alternative to lobectomy.

Endobronchial amyloidosis may cause local obstruction associated with a variety of clinical presentations including recurrent respiratory infection. Although some patients may require surgical intervention, encouraging experience with endobronchial Neodymium (Nd):YAG laser therapy in the management of this condition is reported. Two patients with localized endobronchial amyloidosis were treated with endobronchial Nd:YAG laser therapy. One patient received two treatments for right upper lobe amyloid and another patient received three treatments for right lower lobe amyloid. Excellent clearance of amyloid was achieved in each patient. The first patient remains well, 16 months post-treatment with full patency of the apical and posterior segments. Ten months post-treatment, the second patient has full patency of the right lower lobe. No complications occurred in association with Nd:YAG laser therapy. Endobronchial Neodymium:YAG laser treatment should be considered for selected patients with localized endobronchial obstruction due to amyloidosis.

Airway Obstruction↗
PubMedDate not supplied

Giant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence.

Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloid, characterized by marked hepatomegaly and portal hypertension without hepato-cellular failure and by a severe prognosis, without systemic involvement, is less frequent. The authors describe an unusual case of primary hepatic amyloidosis with giant hepatomegaly, intrahepatic cholestasis, portal hypertension and splenomegaly, occurred in an elderly patient.

Aged↗
PubMedDate not supplied

Cardiac and intestinal amyloidosis in a renal transplant recipient with familial Mediterranean fever.

In Turkey, familial Mediterranean fever (FMF) is an important cause of nephrotic syndrome and endstage renal disease due to renal deposition of AA type amyloid. We report a case of living-related donor renal transplant recipient with FMF and renal AA type amyloidosis, who died of progressive heart failure due to cardiac involvement. The patient also had intractable diarrhea caused by biopsy-proven intestinal amyloidosis. The patient was on 1 mg/day colchicine. Although he was attack-free throughout the post-transplant period, intestinal and clinically significant cardiac amyloidosis, which implied the presence of sustained inflammation and continuing amyloid deposition, appeared three years after renal transplantation. Cardiac deposition of AA amyloid may cause clinically significant heart disease, leading to cardiovascular mortality after renal transplantation for end-stage renal disease in FMF patients.

Adult↗
PubMedDate not supplied

[Retrospective analysis of 44 cases of amyloidosis].

To know the natural history and the several clinical manifestations of amyloidosis, were reviewed retrospectively 44 cases at the Hospital General, Centro Médico Nacional, I.M.S.S. We found the secondary type in 79 per cent, primary shape in 9 per cent and 2 per cent associated with multiple myeloma. The frequency was similar in both sexes and was greater between the fifth and sixth decade of life; 34 per cent of the patients were diabetic; the symptomatology corresponded in 90 per cent of the cases to the main pathology, and only 9.9 per cent of the cases it was secondary to the amyloidosis itself; the alterations of laboratory test were not specific. The organs involved in the cases of primary amyloidosis were: spleen (100 per cent), kidney (80 per cent), heart (80 per cent), digestive tract (60 per cent), and lung (60 per cent). In the secondary shape: spleen (56 per cent), liver (41 per cent), adrenals (23 per cent) and kidney (11 per cent). Death could generally be adscribed to intercurrent pathology.

Adult↗
PubMedDate not supplied

Spontaneous resolution of diffuse alveolar septal amyloidosis.

Amyloidosis is a generic term for a heterogeneous group of disorders associated with deposition of protein in an abnormal fibrillar form. Amyloidosis can be hereditary or acquired, localised or systemic and potentially lethal or merely an incidental finding. We report a case of a male who was totally asymptomatic and was incidentally detected to have lung amyloidosis. He was not given any treatment but kept under observation., His chest radiograph (CXR) and high resolution computed tomograph (HRCT) of the chest cleared spontaneously after one year and the patient continues to remain asymptomatic even after two years.

Adult↗
PubMed2001

[Detection of monoclonal plasma cells in bone marrow and spleen of primary amyloidosis].

A 53-year-old man was diagnosed as primary amyloidosis by biopsy specimens of the mucosa in rectum, spleen and bone marrow (BM). BM examination showed 5.5% of plasma cells with some dysplasia. Immunoglobulin heavy chain gene rearrangement was detected by polymerase chain reaction using third-complementary-determining region (CDR 3) specific primers in BM mononuclear cells and spleen cells embedded in paraffin. The sequence analysis revealed that monoclonal B cells existed in the both, BM and spleen. Flowcytometric analysis using two-color staining showed the phenotype of plasma cells with the expression of CD19+/-, CD27+/-, CD56+/- and CD138+ and CD38++. This phenotype is similar to those of monoclonal gammopathy of undetermined significance (MGUS). Therefore, primary amyloidosis is considered to have two plasma cell populations including normal plasma cells and monoclonal plasma cells in BM. Given that there are two types of plasma cells in patients with primary amyloidosis, amyloid protein is expected to originate from immunoglobulin light chain produced by monoclonal plasma cells.

Amyloid↗
PubMed2001

Multiple myeloma-associated amyloidosis manifesting as fulminant hepatic failure.

We report a case of amyloidosis associated with K light chain multiple myeloma in a 42-year-old African American man. The patient initially had mild dyspepsia, which rapidly progressed to include anorexia, fulminant hepatic failure, and death within 9 weeks. This is only the fourth reported case of hepatic failure from myeloma-associated amyloidosis and the second reported case of light chain myeloma with amyloidosis resulting in a progressive clinical course of hepatic failure. Our patient was unique in that, despite severe disease, he had mild symptoms without laboratory abnormalities until 2 weeks prior to death.

Adult↗
PubMed1998

[Clinicopathological and diagnostic study of amyloidosis of oral maxillofacial area].

OBJECTIVE: To study the clinicopathological features of amyloidosis studied in oral maxillofacial area and the diagnostic methods. METHODS: HE staining and staining of alkaline Congo red, Immunohistochemical staining, Sections examined by transmitted and polarized light microscopy, Transmitted electron microscopy. RESULTS: 14 cases of the disease were reported. Clinically, the patients usually had progressive enlargement and solidness of the tongue (macroglossia, 12 cases), hard nodules or cords of the cheek and lip mucosa (8 cases), single or multiple hard masses of submandibular and submental areas (6 cases), and ecchymosis or petechiae of the face, neck, chest or oral mucosa. Microscopically, the disease is characterized by the even pink amyloid protein deposit in the soft tissues and the wall of blood vessles, which shows positive staining of alkaline Congo red, Immunohistochemical staining can differentiate the primary amyloidosis from secondary one. Ultrastructurally, Amyloid protein appeares as felt-like finely filament. CONCLUSION: Amyloidosis involving in oral maxillofacial area mainly arise in tongue, less frequently, in buccal and lip mucosa, salivary glands. It is not difficulty to dignosis the disease by HE staining combining with staining of alkaline Congo red and other specific staining methods. Ultrastructural and immunohistochemical study is helpful to make show the dignosis.

Adult↗
PubMedDate not supplied

[Systemic amyloidosis secondary to xanthogranulomatous pyelonephritis].

Secondary amyloidosis is a relatively common pathology in which chronic infectious diseases are common causes, especially infected bronchiectasis, osteomyelitis or chronic ulcers. The association of xanthogranulomatous pyelonephritis and systemic amyloidosis is extremely rare. To our knowledge, despite innumerable cases of xanthogranulomatous pyelonephritis reported in the literature, this association has been reported in only 8 previous cases. Patients usually complain of fever, back or flank pain and urinary tract symptoms. A long lasting evolution of the process is frequent. We report a 70 year old patient who developed amyloidosis secondary to xanthogranulomatous pyelonephritis. As well as the rarity of this association, this case is exceptional in its clinical presentation, without any urinary tract symptoms that could suggest the diagnosis.

Aged↗
PubMed2002

[Extensive subcutaneous hemorrhage complicating primary amyloidosis].

Bleeding manifestations are common in patients with primary as well as secondary amyloidosis and have been attributed to different mechanical and metabolic causes. Factor X deficiency has been described in patients with primary amyloidosis, although severe bleeding is not common in these patients. We describe a patient with extensive subcutaneous hematoma, a sign that served as the major indicator assisting us in establishing the diagnosis of primary amyloidosis.

Aged↗
PubMed1999

[The amyloidosis in cardiac conduction system].

OBJECTIVE: To investigate the relationship between arrhythmia, cardiac failure and amyloidosis of cardiac conduction system (CCS). METHODS: CCS from 6 cases with cardiac amyloidosis were sampled using technique designed by the authors. The CCS were studied by Congo red staining and polarization microscopy. RESULTS: Pathological changes could be found in the CCS of these 6 cases. It was serious in sino-atrial node while slight in atrio-ventricular conduction system. The changes in atrio-ventricular conduction system were exacerbated from proximal part (atrio-ventricular node) to distal part (left bundle branch). In the 3 cases with sinus bradycardia and conduction blocking manifested by electrocardiogram, the pathological changes in CCS were more extensive and serious than that of other cases. CONCLUSION: The amyloidosis in CCS is one of the pathological bases of arrhythmia.

Aged↗
PubMedDate not supplied

[Retropancreatic hematoma mimicking a tumor in a female patient with liver amyloidosis].

Bleeding in patients with amyloidosis is considered as a frequent complication appearing in up to 40% of cases. It may be spontaneous or provoked. Spontaneous bleeding in the skin or in the gastrointestinal tract is the most frequent. Spontaneous bleeding in the retroperitoneum is rare. It may be diffuse, with possible lethal outcome, or localised, causing haematoma, as in our patient. We present a 62-year old woman with a three-month history of spontaneous, subcutaneous bleeding. No laboratory or other findings could explain the cause of bleeding, as the haemostatic tests were within or close to normal limits. US and CT performed for right subcostal pain discovered a tumour attached to the back of the head of pancreas. Due to mild coaugated hyperbilirubinaemia the "tumour" was held to be the cause of patient's difficulties. However, at operation it turned out to be a retropancreatic haematoma, which was removed and drained. Because of conjugated hyperbilirubinaemia cholecystectomy with operative cholangiography (which was normal) were performed. Due to steatotic appearance of the liver a biopsy was performed. Histological examination revealed amyloidosis. The recovery was uneventful and the patient was transferred to hepatology unit for further treatment where she died 3 months later due to gradual deterioration of kidney and heart function. To our knowledge, retroperitoneal haematoma in liver amyloidosis mimicking retropancreatic tumour has not been reported so far.

Amyloidosis↗