Adult ; Amyloidosis ; diagnosis ; pathology ; Female ; Humans ; Liver Diseases ; diagnosis ; pathology

Aged ; Amyloidosis ; diagnosis ; pathology ; Humans ; Kidney Pelvis ; pathology ; Male ; Ureter ; pathology ; Urinary Bladder ; pathology

Amyloidosis is classified according to the distribution pattern of amyloid deposition sites and associated diseases. Hepatic amyloidosis is not infrequent, although rarely causes clinical liver disease. We report two cases of amyloidosis diagnosed by liver biopsy. One presented with symptoms related almost to the liver disease, such as jaundice, hepatomegaly and indigestion. Echocardiogram revealed hypertrophic cardiomyopathy, suggesting cardiac involvement of the amyloidosis. The patient died of hepatic failure. The other case was found in a patient with an end stage renal disease. Features of congestive heart failure in this case may reflect cardiac involvement. The pattern of hepatic amyloid deposition in both of these cases was diffuse perisinusoidal. The predominant intralobular deposition suggests that these are amyloidosis of the secondary type.
Amyloidosis/*diagnosis/pathology ; Humans ; Liver Diseases/*diagnosis/pathology ; Male ; Middle Aged

The correlations between the clinicopathological features and the long-term outcomes of renal amyloidosis (RA) were analyzed with a view to develop strategies for improving diagnosis and prognosis of RA. We retrospectively reviewed the clinicopathological characteristics of 47 patients diagnosed with RA between 2004 and 2014 at the Wuhan Union Hospital. The data on the renal histology, clinical manifestations, and prognosis of RA patients were retrieved from the hospital records and characteristic patterns were identified. The histological changes in the kidneys were correlated with the clinical manifestations of RA. Additionally, most RA patients in this study had decreased serum levels of κ light chain and increased urine levels of κ and λ light chains as well as presence of M-protein in the urine and serum. Patients with early RA showed no specific pathognomonic symptoms. Bleeding associated with diagnostic renal biopsy was rare. We recommend that the routine work-up of patients aged over 40 years and presenting with non-diabetic nephropathy includes the non-invasive tests for the measurement of serum and urine levels of κ and λ light chains as well as protein electrophoresis tests for the presence of urinary and serum M-protein. Additionally, such patients should undergo renal biopsy screening with Cong-red staining to ensure early diagnosis of RA and improve their survival, since the risk of hemorrhage related to renal biopsy screening is low at early stages of RA.
Amyloidosis ; diagnosis ; pathology ; Biopsy ; Humans ; Immunoglobulins ; blood ; urine ; Kidney Diseases ; diagnosis ; pathology ; Prognosis

OBJECTIVE: To determine clinical features and diagnostic methods for primary tracheobronchial amyloidosis (TBA).
 METHODS: The clinical manifestations and diagnosis of a male patient who had been misdiagnosed for many years were described and analyzed.
 RESULTS: The patient was a 68-year-old male who complained of recurrent cough, expectoration, and progressive dyspnea for more than 30 years. He had been diagnosed with chronic bronchitis, bronchiectasis, and endobronchial tuberculosis in other hospitals and treated with antibiotics frequently and anti-tubercular agents for 3 months. Despite the treatments, the patient's symptoms were progressively worse. Finally, he came to Xiangya Hospital, Central South University, and was clearly diagnosed with primary TBA based on histopathological evidence after bronchoscopy.
 CONCLUSION TBA, a rare disease resulting from abnormal submucosal amyloid deposition in the trachea and bronchi, may display with many different symptoms. TBA is often misdiagnosed with other pulmonary diseases. The use of bronchoscopic techniques is essential for the diagnosis of TBA. Histopathology remains the gold standard for diagnosis of primary TBA. So, for patients with chronic cough of unknown etiology, bronchoscopy should be performed to obtain biopsy samples for the definitive diagnosis.
Aged ; Amyloidosis ; diagnosis ; Bronchi ; pathology ; Bronchial Diseases ; diagnosis ; Bronchiectasis ; Bronchitis, Chronic ; Bronchoscopy ; Delayed Diagnosis ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Trachea ; pathology ; Tracheal Diseases ; diagnosis ; Tuberculosis

Adult ; Amyloidosis ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Muscular Diseases ; pathology ; surgery ; Neck Muscles ; pathology ; surgery

OBJECTIVETo explore the clinical features of multiple myeloma with different renal pathology, and to evaluate its prognosis.

METHODSClinical features and prognosis of 46 multiple myeloma patients with different renal pathology were analyzed retrospectively. According to renal pathology, the 46 patients were divided into 3 groups: cast nephropathy (24 cases), amyloidosis (15 cases) and other type (7 cases).

RESULTSBy durie-Salmon staging system, 70.8% cases (17/24) in the cast nephropathy group were in Phase III, 90.9% (20/24) were in subtype B, while in amyloidosis group 53.3% (8/15) were in Phase I, 40% (6/15) were in subtype B, and in other types group, 71.4% (5/7) were in phase III, 57.1% (4/7) were in subtype B, the differences among them were statisticaily significant (P < 0.05). In cast nephropathy group, the monoclonal immunoglobulin could not be detected in 75% (18/24) cases, which was light chain type, while immunoglobulin in amyloidosis and other type groups were mainly IgG type in 73.3% (11/15) and 71.4% (5/7) respectively, the difference among them also was statistically significant (P < 0.05). The median survival time of patients in cast nephropathy group was 11 months, while that in amyloidosis and other type groups was 19 and 18 months, the differences among 3 groups were not significant (P > 0.05).

CONCLUSIONIn renal pathologic types, the cast nephropathy is the most common, followed by amylordosis. The multiple mycloma patients with defferent renal pathology show different clinical features. The multiple myeloma patients with renal amyloidosis have slighter clinical manifestations possibly with a better prognosis. Meanwhile, the non-amyloidosis types, especially cast nephropathy may predict a more serious manifications with poor prognosis.

Amyloidosis ; diagnosis ; pathology ; Humans ; Kidney ; pathology ; Kidney Diseases ; diagnosis ; pathology ; Multiple Myeloma ; diagnosis ; pathology ; Prognosis ; Retrospective Studies

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

Objective To evaluate the sensitivities of various biopsy methods for the diagnosis of systematic amyloidosis (SA). Methods The clinical data and biopsy results of 194 SA patients who were treated in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed. Results The highest sensitivity was achieved by biopsy of affected organs,with renal biopsy 97.4%,heart biopsy 95.0% and liver biopsy 87.5%. Among non-invasive biopsy methods,tongue biopsy was found to be 75% sensitive,followed by gingiva biopsy at 57%,abdominal fat pad aspiration at 57%,rectum biopsy at 16%,and bone marrow examination at 8%. Combination of tongue and abdominal fat pad biopsy yielded a detection rate of 93.1%. Conclusions Biopsy of the involved organ has the highest sensitivity. However,combination of multiple non-invasive biopsy methods may has sensitivity comparable to organ biopsy and is safer and more convenient.
Adipose Tissue ; pathology ; Amyloidosis ; diagnosis ; Biopsy ; methods ; Biopsy, Needle ; Humans ; Retrospective Studies ; Sensitivity and Specificity ; Tongue ; pathology

OBJECTIVETo summarize the features of clinical manifestations, laboratory tests and imaging findings of patients with cardiac amyloidosis (CA).

METHODSA total of 60 CA patients (including 41 male and 19 female patients) from 4 centers admitted between May, 2012 and November, 2013 were included in the study. The demographic data, medical history, clinical manifestations, laboratory test data, ECG, cardiac ultrasound, and cardiac magnetic resonance (CMR) imaging of the patients were analyzed.

RESULTSTwo-thirds of the 60 CA patients, were middle-aged or elderly men, and 47% of the patients had AL-CA. The clinical manifestations included exertional dyspnea (73%), pedal edema (47%), hypotension (47%), and hypertrophy of the tongue (22%); abnormal laboratory test results included albuminuria (53%) and liver (15%) and kidney (28%) dysfunction; blood routine, urine and serum immunoglobulin quantification and immunofixation electrophoresis could help the screening of AL-CA. Kidney (53%) and liver (15%) involvement was common, and 86% of AL-CA patients had kidney involvement. Typical ECG characteristics included poor R wave progression (35%), low voltage in limb leads (33%), and a pseudo infarct Q wave (30%); the latter two were more frequent in AL-CA. The characteristics of ultrasound findings included left ventricle thickening (100%), left atrial enlargement (87%) and enhanced echo of the myocardial granules(92%), and diastolic dysfunction was obvious in all the CA patients regardless of the systolic function. The DT and E/e' of the mitral annulus could be used as an index to evaluate diastolic dysfunction in early stage of the disease. Left ventricular (LV) global subendocardial late gadolinium enhancement (LGE, 81%) accompanied by right ventricular (RV) and atrial LGE was the typical characteristic of CMR, and the range of LGE in the RV and the two atria was wider in AL-CA than in non-AL-CA. NT-proBNP (97%) and cardiac troponin (53%) in CA patients were both elevated, which helped in diagnosing and assessing the severity of cardiac involvement, according to which 50% of the patients were found to be at a high risk, 43% at an intermediate risk, and 7% at a low risk.

CONCLUSIONThe combination of the features of clinical, laboratory tests and imaging findings of CA have important diagnostic and prognostic value for CA.

Adult ; Aged ; Amyloidosis ; diagnosis ; pathology ; physiopathology ; Cardiomyopathies ; diagnosis ; pathology ; physiopathology ; Electrocardiography ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Magnetic Resonance Imaging ; Male ; Middle Aged