Kidney Transplantation in Patients With AA Amyloidosis: Outcomes in a French Multicenter Cohort.

RATIONALE & OBJECTIVE: Outcomes of kidney transplantation for patients with renal AA amyloidosis are uncertain, with reports of poor survival and high rates of disease recurrence. However, the data are inconclusive and mostly based on studies from the early 2000s and earlier. STUDY DESIGN: Retrospective multicenter cohort study. SETTING & PARTICIPANTS: We searched the French national transplant database to identify all patients with renal AA amyloidosis who underwent kidney transplantation between 2008 and 2018. EXPOSURES: Age, cause of amyloidosis, use of biotherapies, and C-reactive protein levels. OUTCOMES: Outcomes were all-cause mortality and allograft loss. We also reported amyloidosis allograft recurrence, occurrence of acute rejection episodes, as well as infectious, cardiovascular, and neoplastic disease events. ANALYTICAL APPROACH: Kaplan-Meier estimator for mortality and cumulative incidence function method for allograft loss. Factors associated with patient and allograft survival were investigated using a Cox proportional hazards model and a cause-specific hazards model, respectively. RESULTS: 86 patients who received kidney transplants for AA amyloidosis at 26 French centers were included. The median age was 49.4 years (IQR, 39.7-61.1). The main cause of amyloidosis was familial Mediterranean fever (37 cases; 43%). 16 (18.6%) patients received biotherapy after transplantation. Patient survival rates were 94.0% (95% CI, 89.1-99.2) at 1 year and 85.5% (77.8-94.0) at 5 years after transplantation. Cumulative incidences of allograft loss were 10.5% (4.0-17.0) at 1 year and 13.0% (5.8-20.1) at 5 years after transplantation. Histologically proven AA amyloidosis recurrence occurred in 5 transplants (5.8%). An infection requiring hospitalization developed in 55.8% of cases, and there was a 27.9% incidence of acute allograft rejection. Multivariable analysis showed that C-reactive protein concentration at the time of transplantation was associated with patient survival (HR, 1.01; 95% CI, 1.00-1.02; P=0.01) and allograft survival (HR, 1.68; 95% CI, 1.10-2.57; P=0.02). LIMITATIONS: The study lacked a control group, and the effect of biotherapies on transplantation outcomes could not be explored. CONCLUSIONS: This relatively contemporary cohort of patients who received a kidney transplant for AA amyloidosis experienced favorable rates of survival and lower recurrence rates than previously reported. These data support the practice of treating these patients with kidney transplantation for end-stage kidney disease. PLAIN-LANGUAGE SUMMARY: AA amyloidosis is a severe and rare disease. Kidney involvement is frequent and leads to end-stage kidney disease. Because of the involvement of other organs, these patients are often frail, which has raised concerns about their suitability for kidney transplantation. We reviewed all patients with AA amyloidosis nephropathy who underwent kidney transplantation in France in the recent era (2008-2018) and found that the outcomes after kidney transplantation were favorable, with 85.5% of patients still alive 5 years after transplantation, a survival rate that is comparable to the outcomes of patients receiving a transplant for other forms of kidney diseases. Recurrence of amyloidosis in the transplanted kidney was infrequent (5.8%). These data support the practice of kidney transplantation for patients with AA amyloidosis who experience kidney failure.

Amyloidosis and Considerations for the Hand Surgeon.

Amyloidosis can lead to cardiac, renal, and other multiorgan failure. New treatments have become available that can prolong survival but rely on early diagnosis. Manifestations of amyloidosis in hand surgery include carpal tunnel syndrome, trigger finger, peripheral neuropathy, and spontaneous distal biceps rupture. Often, these can predate systemic amyloidosis, offering hand surgeons an opportunity to diagnose patients with amyloidosis before systemic disease, refer them for treatment, and potentially alter disease course and prolong survival. In this review, we describe the pathophysiology and two most common subtypes of amyloidosis seen by hand surgeons. We provide guidance on biopsy practices and referral for patients with amyloidosis. Lastly, we provide a brief overview of the treatments for amyloidosis and their effect on disease course.

Being a Better Starfish Thrower, Carpal Tunnel Syndrome, and Cardiac Amyloidosis: The Hand Surgeon's Opportunity to Make a Profound Difference.

In recent years, there is increasing literature in cardiac and hand surgery journals demonstrating a stronger association between seemingly idiopathic carpal tunnel and amyloidosis. Despite this, it can be difficult for hand surgeons to identify who need biopsies, and this is further complicated by the cost of a biopsy and the low likelihood that a patient has cardiac amyloidosis. In patients with cardiac amyloidosis and carpal tunnel syndrome (CTS), CTS is typically diagnosed 5-10 years prior. Early diagnosis of cardiac amyloidosis is crucial, as current medications work to slow disease progression, but do not treat existing amyloid deposits. Hand surgeons can play an essential role in early diagnosis. The patient case discussed describes a man who had a carpal tunnel biopsy because of his bilateral CTS, recurrent trigger fingers, and his age. After confirmation of amyloidosis, he was referred for cardiac amyloidosis evaluation. Testing confirmed this diagnosis, and he was started on tafamidis, which studies show provide patients an opportunity for increased survival and quality of life. The responsibility falls on cardiologists and hand surgeons to continue refining the indications for carpal tunnel biopsy and spreading awareness of carpal tunnel biopsy and amyloid testing, as much work is still needed.

[A Case of Transverse Colon Cancer with Gastrointestinal Amyloidosis Prevented from Anastomotic Leakage by Multidimensional Approach].

The patient was a 68-year-old woman who was on hemodialysis due to systemic amyloidosis and nephrotic syndrome. Biopsy revealed amyloid deposition in the stomach, duodenum, and colon. A transverse colon tumor was found on a follow- up CT after the aortic dissection surgery. We performed lower gastrointestinal endoscopy and contrast-enhanced CT and diagnosed transverse colon cancer with gastric wall infiltration(cStage Ⅲc). We considered that transverse colon resection was oncologically sufficient. However, due to concurrent gastrointestinal amyloidosis, which increased the risk of anastomotic leakage we performed laparoscopic extended right hemicolectomy to avoid colon-colon anastomosis with partial gastrectomy. Additionally intraoperative indocyanine green(ICG)fluorescence imaging showed that the fluorescence signal in the small intestinal wall was satisfactory, while it was weak in the colon wall. As a result, we suspected of impaired blood flow of colon wall due to an amyloidosis, so we additionally created a loop ileostomy. It is said that gastrointestinal amyloidosis raises the risk of anastomotic leakage. A case of transverse colon cancer complicated by gastrointestinal amyloidosis in which we successfully prevented anastomotic leakage through a multidimensional evaluation and approach is reported, along with a literature review.

Long-term results of kidney transplantation in patients with familial mediterranean fever.

INTRODUCTION: Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT. METHODS: We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Baskent University Hospital. All data were recorded retrospectively from patients' files. RESULTS: THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up. CONCLUSION: We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.

Surgical treatment of laryngeal amyloidosis: a systematic review.

PURPOSE: The aim of this review was to study the surgical management of laryngeal amyloidosis and estimate the rate of recurrence after surgery. METHODS: A systematic review searching PubMed and EMBASE was performed. A qualitative synthesis of data regarding the surgical management of LA and a quantitative analysis of the recurrence rate after surgery was conducted. RESULTS: This systematic review included 14 retrospective studies, one of whom is retrospective controlled. A total of 515 subjects were included, the mean age ranged from 43.3 to 58 years with a male-to-female ratio of 1:1.3. All cases had a localized laryngeal amyloidosis. The supraglottic region was the most affected laryngeal site and multiple sites were commonly involved. Surgical treatment consists of endoscopic excision using laser, cold or powered instruments. Open surgery is required for severe primary case or revision surgery. Surgical complications such as granulomatosis scar tissue formation, tracheostomy, laryngotracheal stenosis, pneumothorax and concomitant malignancy were developed in 17.5% of patients. The time onset to diagnosis varied from 1 months to 15 years and the duration of follow-up from 3 months to 25 years. The rate of recurrence was 28.4% (95% CI 24.5-32.6) and the timing of recurrences ranged from 3 months to 10 years. CONCLUSION: The recurrence rate after primary surgery for laryngeal amyloidosis is high. A tailored surgical treatment based on the disease extension and a long-term follow up are recommended.

Systemic amyloidosis as a cause of chronic diarrhea after gastric surgery. Literature review.

Amyloidosis with gastrointestinal involvement is a rare cause of chronic diarrhoea, and should be considered especially in adult patients with intestinal malabsorption and extra GI manifestations. We present the case of a male patient who, after an oncological gastrectomy, presented with chronic diarrhea that did not respond to treatment and, after study, the cause of the diarrhea was finally found. Primary systemic light chain amyloidosis (AL) initially presents as chronic diarrhea and weight loss after gastrectomy. Immunohistochemistry was completely negative for amyloid A, which virtually rules out AA amyloidosis. With regard to the immunoglobulin chains, an amyloid signal was observed for both light chains, with a predominance of lambda light chain but not entirely conclusive. This situation is not uncommon since amyloid, whatever its chemical nature, can annex serum proteins, including immunoglobulin chains. In the case of chronic diarrhea, the possibility of amyloidosis should be kept in mind, especially in the case of weight loss.

Outcomes of kidney transplantation in patients with myeloma and amyloidosis in the USA.

BACKGROUND: Recent improvement in treatment and patient survival has opened the eligibility of kidney transplantation to patients who developed end-stage kidney disease (ESKD) from plasma cell dyscrasias (PCDs). Data on clinical outcomes in this population are lacking. METHODS: We conducted a retrospective study of United Network for Organ Sharing/Organ Procurement and Transplantation Network dataset (2006-2018) to compare patient and graft outcomes of kidney transplant recipients with ESKD due to PCD versus other causes. RESULTS: Among 168 369 adult first kidney transplant recipients, 0.22-0.43% per year had PCD as the cause of ESKD. The PCD group had worse survival than the non-PCD group for both living and deceased donor types {adjusted hazard ratio [aHR] 2.24 [95% confidence interval (CI) 1.67-2.99] and aHR 1.40 [95% CI 1.08-1.83], respectively}. The PCD group had worse survival than the diabetes group, but only among living donors [aHR 1.87 (95% CI 1.37-2.53) versus aHR 1.16 (95% CI 0.89-1.2)]. Graft survival in patients with PCD were worse than non-PCD in both living and deceased donors [aHR 1.72 (95% CI 1.91-2.56) and aHR 1.30 (95% CI 1.03-1.66)]. Patient and graft survival were worse in amyloidosis but not statistically different in multiple myeloma compared with the non-PCD group. CONCLUSION: The study data are crucial when determining kidney transplant eligibility and when discussing transplant risks in patients with PCD.

Cervical amyloidoma of transthyretin type: a case report and review of literature.

BACKGROUND: Amyloidoma is a rare clinical entity characterized by the focal aggregation of amyloid protein within the body, void of systemic involvement. To our knowledge, there have only been 26 reports of cervical amyloidoma to date. Amyloid light chain and beta-2-microglobulin are the most common types, with only three previous reports of transthyretin (ATTR) Amyloidoma. CASE PRESENTATION: We report a case of a 71-year-old male who presented with worsening strength and coordination of his upper extremities, right upper-leg pain, unsteady gait, and a reduced range of motion of his neck in all planes. Magnetic resonance imaging revealed a solitary mass compressing the spinal cord at C1-C2. Treatment consisted of cervical decompression and stabilization. Pathological examination confirmed solitary amyloid deposition of ATTR. Postoperative neurological assessment revealed improved balance, gait, hand function, and grip strength. Investigational imaging was ordered 8 months postoperatively revealing no evidence of systemic involvement, confirming the diagnosis of cervical ATTR amyloidoma. A discussion is provided surrounding the published literature of ATTR amyloidoma with description of the typical presentation, management, and outcomes of this rare pathology. CONCLUSION: Previous cases and studies indicate clinical signs such as ligamentum of flavum hypertrophy and carpal tunnel syndrome may precede focal ATTR spinal disposition. Outcomes for amyloidoma are generally favourable, as tumour resection prevents irreversible deficits. Patients have a low rate of recurrence with an overall excellent prognosis following resection and stabilization.

Prevalence and Subtypes of Tenosynovial Amyloid in Patients Undergoing Carpal Tunnel Release.

PURPOSE: The purpose of this study was to determine the prevalence and subtypes of amyloid in tenosynovial biopsies of patients undergoing carpal tunnel release (CTR). METHODS: A retrospective review was performed involving patients who underwent CTR from June 2020 to July 2021. Prior to this period, a protocol had been established to obtain routine intraoperative tenosynovial biopsies. Tenosynovium was preserved in formalin and stained with Congo red for amyloid. Positive specimens were sent to an external laboratory for confirmation and subtyping by mass spectrometry. Men 50 years or older and women 60 or older were included for analysis. Acute, traumatic, and revision cases were excluded. RESULTS: Of 185 patients who underwent CTR with tenosynovial biopsy, 54 (29%) demonstrated positive Congo red stain, confirmed by the external laboratory. A subtype analysis revealed wild-type transthyretin (TTR) in 44 patients (80%), mixed wild-type TTR with κ light chains in 1 patient, and hereditary TTR in 1 patient. Patients with positive specimens were significantly older than those who tested negative (77 vs 68 years, respectively), and positivity increased by decade for both sexes. Male sex, atrial fibrillation, and spinal stenosis were significantly more prevalent among positive cases. There were no complications from the biopsies. CONCLUSIONS: We confirmed evidence of amyloidosis in the tenosynovium of 29% of men 50 years or older and women 60 or older who underwent CTR. The majority demonstrated wild-type TTR. As these patients are at risk of developing cardiomyopathy, there is an opportunity for early detection, monitoring, and interventions known to improve outcomes. Considering the low cost of Congo red staining and the potential value of subtyping with regard to treatment of cardiomyopathy, our findings support routine tenosynovial biopsy during CTR in patients who meet the age criteria. TYPE OF STUDY/LEVEL OF EVIDENCE: Differential diagnosis or symptom prevalence study II.

A study from The Mayo Clinic evaluated long-term outcomes of kidney transplantation in patients with immunoglobulin light chain amyloidosis.

Longer survival using modern therapies has increased the number of patients with immunoglobulin light-chain amyloidosis receiving kidney transplantation. We evaluated 60 patients with immunoglobulin light chain amyloidosis who underwent kidney transplantation based on their hematologic response for outcomes of death, graft failure, and complications. Patient hematologic responses (light-chain in blood or urine) prior to kidney transplantation were three patients had no response, five had a partial response, six had a very good partial response, 37 had a complete response, and nine were treatment-naive patients (never treated for this disorder). After transplantation, seven of nine treatment-naive patients achieved a complete response. The median follow-up for the entire transplant cohort was 61 months. The estimated median overall survival from the time of kidney transplantation was 123 months for the entire group. Median overall survival was not reached for the very good partial response plus complete response groups, it was 47 months for no response plus partial response groups, and 117 months for the treatment-naive group (all significantly different). Median overall survival of very good partial response was 81 months, while the median was not reached in the complete response group (no significant difference). The time to amyloid recurrence was significantly longer in complete response compared to very good partial response (median 181 vs 81 months). Death-censored graft survival at one- and five-years was 98.3%, and 95.8%, respectively for all groups. Of the 60 patients, three had allograft failure, 19 died with a functioning graft, and 13 had an amyloid recurrence. Thus, outcomes after kidney transplant in patients with immunoglobulin light-chain amyloidosis seem acceptable if a very good partial response or complete response is achieved either before or after transplantation.

Renal transplant outcomes in amyloidosis.

BACKGROUND: Outcomes after renal transplantation have traditionally been poor in systemic amyloid A (AA) amyloidosis and systemic light chain (AL) amyloidosis, with high mortality and frequent recurrent disease. We sought to compare outcomes with matched transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) and diabetic nephropathy (DN), and identify factors predictive of outcomes. METHODS: We performed a retrospective cohort study of 51 systemic AL and 48 systemic AA amyloidosis patients undergoing renal transplantation. Matched groups were generated by propensity score matching. Patient and death-censored allograft survival were compared via Kaplan-Meier survival analyses, and assessment of clinicopathological features predicting outcomes via Cox proportional hazard analyses. RESULTS: One-, 5- and 10-year death-censored unadjusted graft survival was, respectively, 94, 91 and 78% for AA amyloidosis, and 98, 93 and 93% for AL amyloidosis; median patient survival was 13.1 and 7.9 years, respectively. Patient survival in AL and AA amyloidosis was comparable to DN, but poorer than ADPKD [hazard ratio (HR) = 3.12 and 3.09, respectively; P < 0.001]. Death-censored allograft survival was comparable between all groups. In AL amyloidosis, mortality was predicted by interventricular septum at end diastole (IVSd) thickness >12 mm (HR = 26.58; P = 0.03), while survival was predicted by haematologic response (very good partial or complete response; HR = 0.07; P = 0.018). In AA amyloidosis, recurrent amyloid was associated with elevated serum amyloid A concentration but not with outcomes. CONCLUSIONS: Renal transplantation outcomes for selected patients with AA and AL amyloidosis are comparable to those with DN. In AL amyloidosis, IVSd thickness and achievement of deep haematologic response pre-transplant profoundly impact patient survival.

Improving contemporary outcomes following heart transplantation for cardiac amyloidosis.

BACKGROUND: The incidence of systemic amyloidosis is rising, and there is a concomitant rise in heart transplant for an indication of cardiac amyloidosis. METHODS: We utilized the Organ Procurement and Transplantation Network (OPTN) database to retrospectively assess survival and outcomes in adult patients undergoing heart transplant for cardiac amyloidosis from 1999 to 2019. We also compared survival among four distinct time periods: 1999-2001, 2002-2008, 2008-2015, 2016-2019. RESULTS: Of 41,103 patients, 425 (1.03%) were transplanted for an indication of restrictive cardiomyopathy due to cardiac amyloidosis (RCM-Amyloidosis). The percent of all transplants occurring for RCM-Amyloidosis increased from 0.25% in the 1999-2001 era to 1.74% in the 2015-2019 era (p < .001). Across eras, Kaplan-Meier survival functions were comparable between RCM-Amyloidosis and non-RCM patients at 1 year (88% vs. 89%, p = .56) and at 5 years (72% vs. 77%, p = .092), but worse for RCM-Amyloidosis patients at 10 years (44% vs. 59%, p = .002). With adjustment for other clinical variables in multivariable Cox regression model, RCM-Amyloidosis was not associated with increased risk of death at 1 year (hazard ratio [HR] = 1.11, p = .56) or at 5 years (HR = 1.20, p = .18), but it was associated with increased risk of death at 10 years (HR = 1.35, p = .01). Cardiac amyloidosis was not associated with any morbidity outcomes following transplant, including graft failure, acute rejection, or hospitalization for infection or rejection. CONCLUSIONS: Our data suggest a trend of improving survival among RCM-Amyloidosis patients compared with non-RCM patients across transplant eras, with current similarities in 1- and 5-year survival but a persistent, increased risk of mortality at 10 years.

[Primary Pulmonary Amyloidosis with a Localized Consolidation:Report of Two Cases].

We experienced two cases of primary pulmonary amyloidosis with a localized consolidation. Case 1 is a 80-year-old man, who was found to have an abnormal chest nodular shadow with blurred margin at a medical examination. Chest computed tomography( CT) showed a localized consolidation on the periphery of the upper lobe of the right lung. A CT-guided biopsy was performed. Case 2 is a 66-year-old woman, who was found to have an abnormal chest opacity at a medical examination. Chest CT showed a localized gathering of small nodules in the right lower lobe. Gradual enlargement was noted by follow up CT and the accumulation of fluorodeoxyglucose (FDG) was shown by PET/CT. In consideration of primary lung cancer or malignant lymphoma, right lower lobectomy was performed. Both cases were pathologically diagnosed as pulmonary amyloidosis. Since no findings of amyloid deposits in other organs or of existence of any blood disorders, a diagnosis of primary pulmonary amyloidosis was made.

Catheter ablation of atrial fibrillation in cardiac amyloidosis.

BACKGROUND: Cardiac amyloidosis is a progressive infiltrative disease involving deposition of amyloid fibrils in the myocardium and cardiac conduction system that frequently manifests with heart failure (HF) and arrhythmias, most frequently atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT). METHODS: We performed an observational retrospective study of patients with a diagnosis of cardiac amyloid who underwent catheter ablation at our institution between January 1, 2011 and December 1, 2018. Patient demographics, procedural characteristics, and outcomes were determined by manual chart review. RESULTS: A total of 13 catheter ablations were performed over the study period in patients with cardiac amyloidosis, including 10 AT/AF/AFL ablations and three atrioventricular nodal ablations. Left ventricular ejection fraction was lower at the time of AV node ablation than catheter ablation of AT/AF/AFL (23% vs 40%, P = .003). Cardiac amyloid was diagnosed based on the results of preablation cardiac MRI results in the majority of patients (n = 7, 70%). The HV interval was prolonged at 60 ± 15 ms and did not differ significantly between AV nodal ablation patients and AT/AF/AFL ablation patients (69 ± 18 ms vs 57 ± 14 ms, P = .36). The majority of patients undergoing AT/AF/AFL ablation had persistent AF (n = 7, 70%) and NYHA class II (n = 5, 50%) or III (n = 5, 50%) HF symptoms, whereas patients undergoing AV node ablation were more likely to have class IV HF (n = 2, 66%, P = .014). Arrhythmia-free survival in CA patients after catheter ablation of AT/AF/AFL was 40% at 1 year and 20% at 2 years. CONCLUSIONS: Catheter ablation of AT/AF/AFL may be a feasible strategy for appropriately selected patients with early to mid-stage CA, whereas AV node ablation may be more appropriate in patients with advanced-stage CA.

Amyloidoma of the Tongue: Case Report, Surgical Management, and Review of the Literature.

PURPOSE: Localized amyloidosis of the tongue is a benign condition in which surgical management may be considered. The aim of the study was to review the current literature and report a case. MATERIALS AND METHODS: We searched the PubMed database for all relevant articles reporting cases of localized tongue amyloidosis published between 1980 and February 2020. In addition, we updated 1 case diagnosed and treated in our department. RESULTS: A 49-year-old male patient presented with an asymptomatic tongue nodule of the dorsum mimicking median rhomboid glossitis. The results of an incisional biopsy showed an amyloid on Congo red staining and positive findings for the κ light chain by immunohistochemical analysis. The findings of the systemic workup were negative. Therefore, a diagnosis of localized κ light-chain amyloidosis was made. The patient underwent a resection of the lesion, and no recurrence or progression was observed during a period of 18 months. The literature review showed 12 reports describing 21 patients (11 men, 52.3%) with localized tongue amyloidosis. The most common clinical presentation was nodular with a single lesion of the tongue dorsum (15 patients, 71.4%). All cases showed positive findings on Congo red staining. Immunohistochemical analysis findings were available for only 9 patients (42.8%) and showed light-chain amyloidosis. No case showed any systemic involvement or the development of systemic disease. Surgical excision was performed in 9 cases, with recurrence at the site of operation in 2 cases. CONCLUSIONS: Localized amyloidosis of the tongue is a rare disease in which surgical excision may be therapeutic when a multidisciplinary evaluation does not show any systemic disease. We recommend an excision when the lesion is persistent or shows an enlargement or when discomfort is reported. In the case of any further local recurrence, resection may be repeated.

Coronary revascularisation in cardiac amyloidosis.

We present a case of coronary artery bypass grafting in a 78-year-old man with triple vessel disease and concomitant cardiac amyloidosis. Postoperatively, he developed a profound low cardiac output state and multiorgan failure. He died 3 weeks following surgery. Bypass surgery is rarely performed in patients with cardiac amyloidosis, and there is little in the literature regarding outcomes. The few published cases present a bleak picture, and hence percutaneous coronary intervention should always be preferred.

Risk factors for recurrence of laryngeal amyloidosis treated by microforceps and CO2 laser.

INTRODUCTION: Laryngeal amyloidosis is a benign, slowly progressive disease. The factors affecting the recurrence of LA have not been studied before for the rarity and incomplete understanding of this disease. To investigate the risk factors for the laryngeal amyloidosis treated by microforceps or carbon dioxide laser under microlaryngoscope, a retrospective review was conducted. MATERIALS AND METHODS: One hundred and four patients (42 male and 72 female, with an average age of 51.3 years) with laryngeal amyloidosis were identified. The cases were collected from January 1989 to May 2014 at the Eye, Ear, Nose, and Throat Hospital of Fudan University. RESULTS: Seventy-five patients complained of hoarseness, 36 patients complained of hoarseness and dyspnea, and 3 patients complained of foreign body sensation. All patients underwent surgical removal of the amyloid deposits (via microforceps, CO2 laser and tracheotomy). Thirty patients developed recurrences requiring further treatments. The duration from onset to the treatment and age affected the recurrence of laryngeal amyloidosis, the surgery method, stenotic degree and stenotic area of subglottic area and trachea did not affect the recurrence of the disease. CONCLUSION: Early diagnosis and treatment of laryngeal amyloidosis may reduce the recurrence of the disease. Regular follow-up is necessary to find any recurrence.

Recurrent breast amyloidosis associated with Sjögren's syndrome: A case report with description of the radiologic-pathologic correlation.

We describe the case of a 41-year-old woman with primary Sjögren's syndrome (SS) who presented multiple recurrences of breast amyloidosis. Each recurrence of breast amyloidosis showed different sonographic features, potentially mimicking malignancy. We briefly discuss the possible cause of this variability in imaging features based on the radiologic-histologic correlation.

[Orbital Amyloidosis - Comparison of Two Different Clinical Courses]. / Orbitale Amyloidose ­ Vergleich zweier unterschiedlicher klinischer Verläufe.

BACKGROUND: Primary localised orbital amyloidosis (PLOA) is a very rare disease. In contrast to the isolated manifestation, systemic involvement can be associated with potentially life-threatening consequences. However, the isolated involvement of the orbit can also lead to serious complications. MATERIAL AND METHODS: Two cases of PLOA are described and the necessary ophthalmic, internistic and immunohistochemical diagnostic testing are explained. RESULTS: The first case describes a 71-year-old woman with PLOA. In the clinic, a yellow-orange bumpy prominence in the nasal lower quadrant without further ophthalmological abnormalities was found. Extensive diagnostic testing found no systemic manifestation. The patient herself was free of complaints. In the follow-up over 4 years, patient showed slow progression without ocular complications. The second case is a 72-year-old male patient with similar clinical signs but localisation in the temporal superior quadrant. During the clinical course, multiple ophthalmological complications developed (ptosis, protrusio bulbi, diplopia, secondary glaucoma, perforated corneal ulcer in neurotrophic keratopathy). Perforating keratoplasty had to be performed. Fractioned radiotherapy led to stabilisation of the disease. The follow-up period was 4 years. CONCLUSION: PLOA can lead to visual and organ threatening complications. Accurate diagnosis is required for further diagnostic and therapeutic procedures and to counteract potential local and systemic complications. Interindividual differences in the course have to be considered.