Humans ; Amyloidosis/pathology*

Objective: To investigate the histological features and clinical manifestations in different types of cardiac amyloidosis to improve diagnostic accuracy. Methods: The histopathological features and clinical manifestations of 48 patients diagnosed with cardiac amyloidosis by Congo red stain and electron microscopy through endomyocardial biopsy were collected in West China Hospital of Sichuan University from January 2018 to December 2021. Immunohistochemical stains for immunoglobulin light chains (κ and λ) and transthyretin protein were carried out, and a review of literature was made. Results: The patients age ranged from 42 to 79 years (mean 56 years) and the male to female ratio was 1.1 to 1.0. The positive rate of endomyocardial biopsy was 97.9% (47/48), which was significantly higher than that of the abdominal wall fat (7/17). Congo red staining and electron microscopy were positive in 97.9% (47/48) and 93.5% (43/46), respectively. Immunohistochemical stains showed 32 cases (68.1%) were light chain type (AL-CA), including 31 cases of AL-λ type and 1 case of AL-κ type; 9 cases (19.1%) were transthyretin protein type (ATTR-CA); and 6 cases (12.8%) were not classified. There was no significant difference in the deposition pattern of amyloid between different types (P>0.05). Clinical data showed that ATTR-CA patients had less involvement of 2 or more organs and lower N-terminal pro-B-type natriuretic peptide (NT-proBNP) than the other type patients (P<0.05). The left ventricular stroke volume and right ventricular ejection fraction of ATTR-CA patients were better than the other patients (P<0.05). Follow-up data of 45 patients was obtained, and the overall mean survival time was 15.6±2.0 months. Univariate survival analysis showed that ATTR-CA patients had a better prognosis, while cardiac amyloidosis patients with higher cardiac function grade, NT-proBNP >6 000 ng/L, and troponin T >70 ng/L had a worse prognosis (P<0.05). Multivariate survival analysis showed that NT-proBNP and cardiac function grade were independent prognostic factors for cardiac amyloidosis patients. Conclusions: AL-λ is the most common type of cardiac amyloidosis in this group. Congo red staining combined with electron microscopy can significantly improve the diagnosis of cardiac amyloidosis. The clinical manifestations and prognosis of each type are different and can be classified based on immunostaining profile. However, there are still a few cases that cannot be typed; hence mass spectrometry is recommended if feasible.
Humans ; Male ; Female ; Adult ; Middle Aged ; Aged ; Prealbumin/metabolism* ; Stroke Volume ; Cardiomyopathies/pathology* ; Congo Red ; Ventricular Function, Right ; Amyloidosis/pathology* ; Prognosis

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*

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

OBJECTIVETo evaluate the clinical characteristics of multiple myeloma (MM) combined with renal amyloidosis and its curative efficacy and prognosis.

METHODSThe clinical data of 22 cases of newly diagnosed multiple myeloma combined with renal amyloidosis treated in our hospital from November 2011 to July 2015 were analyzed retrospectively.

RESULTSAccording to Intenational Staging System (ISS), among above-menthioned 22 patients the ISS II accounted for 77.2% (17/22), ISS III accounted for 22.8% (5/22). The patients with renal impairment accounted for 36.4% (8/22), with anemia 40.9% (9/22), with serum album < 35 g/L 86.4% (19/22), with urinary protein positive 100% (22/22). The evaluation of the curative efficacy of the 22 cases was as follows: CR 13.6% (3/22); VGPR 4.5% (1/22); PR 22.8% (5/22); SD 45.5% (10/22); PD 13.6% (3/22). Out of 9 patients with effective treatment, 3 cases (3/9, 33.3%) achieved "improved" in renal amyloidosis, 4 cases (4/9, 44.5%) achieved stable in renal amyloidosis, 2 cases (2/9, 2%) achieved "worsened" in renal amyloidosis. Among 17 cases who were followed up, 7 cases died, 10 cases survived, the average duration of follow-up for these cases was 11 (1-37) months, the median overall survival (OS) time was 19 (95% CI 9.2-28.8) months.

CONCLUSIONMM with renal amyloidosis is rare, refractory and has a poor prognosis. Whether there is impairment of kidney function or not, renal amyloidosis shall be taken into consideration if the MM patients got massive proteinuria especially nephritic syndrome. Bortezomib may improve the curative efficacy.

Amyloidosis ; diagnosis ; pathology ; therapy ; Bortezomib ; therapeutic use ; Humans ; Kidney Diseases ; diagnosis ; pathology ; therapy ; Multiple Myeloma ; diagnosis ; pathology ; therapy ; Prognosis ; Proteinuria ; diagnosis ; Retrospective Studies ; Treatment Outcome

We report a case of inadvertent hoarseness after surgery for primary pulmonary amyloidosis. A 55-year-old male was transferred to our facility due to a lung mass. Chest computed tomography revealed a solitary pulmonary nodule. Positron emission tomography-computed tomography showed fluorodeoxyglucose uptake in the main mass and in the mediastinal lymph nodes. To confirm the pathology of the mass, wedge resection and thorough lymph node dissection were performed via video-assisted thoracic surgery (VATS). No complications except for hoarseness were observed; hoarseness developed soon after surgery and lasted for 3 months. The main mass was diagnosed as amyloidosis, but this was not found in the lymph nodes. In conclusion, VATS wedge resection for peripheral amyloidosis is a feasible and safe procedure. However, mediastinal lymph node dissection is not recommended unless there is evidence of a clear benefit.
Amyloidosis* ; Electrons ; Hoarseness ; Humans ; Lung ; Lymph Node Excision ; Lymph Nodes ; Lymphatic Diseases* ; Male ; Middle Aged ; Pathology ; Solitary Pulmonary Nodule ; Thoracic Surgery, Video-Assisted ; Thorax

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

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

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 summarize the specific CT characteristics and the endoscopic findings of primary tracheobronchial amyloidosis (PTBA) for improvement of the diagnostic accuracy.
 METHODS: The imaging features of 6 patients with PTBA were analyzed by multiplanar reconstructed CT and the fiberoptic bronchoscope, and the pathology were summarized retrospectively.
 RESULTS: All PTBA patients received bronchoscopic examination and the definite diagnosis were confirmed by positive staining with Congo red. PTBA presented diffuse thickening of major airway and lumen stenosis in various degrees with scattered hemorrhage of the mucous membrane under CT and bronchoscope, which was more obvious in low part of trachea, main bronchus and lobar bronchus. The mucosa of trachea and bilateral main bronchi were irregular and bumpy with jutting nodes in 5 patients, which was called "wavy path" pattern. Widely nodular or stripy calcifications of airway were found in 4 patients, which was considered as specific imaging features in PTBA and was involved bilateral main bronchi largely. There were obstructive atelectasis in 2 patients, and calcifications of hilus of lung with longitudinal diaphragm lymph nodes in 3 patients, but they were not specific. Ignoring the extensive circumferential thickening of large airway, "wavy path sign" and rail-like calcification was mainly responsible for misdiagnosis of PTBA as endobronchial tuberculosis or other diseases.
 CONCLUSION Attentions to the specific imaging features on multiplanar CT and the endoscopic findings are the fundamentals to avoid the misdiagnosis of PTBA.
Amyloidosis ; diagnosis ; pathology ; Bronchi ; pathology ; Bronchial Diseases ; diagnosis ; pathology ; Bronchoscopy ; Calcinosis ; Constriction, Pathologic ; Diagnostic Errors ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Lung ; pathology ; Pulmonary Atelectasis ; Retrospective Studies ; Tomography, X-Ray Computed ; Trachea ; pathology