Amyloidosis Presenting Research Articles

Cutaneous insulin-derived amyloidosis presenting as hyperkeratotic nodules.

Amyloidosis consists of approximately 30 protein-folding disorders in which a specific soluble precursor protein aggregates to form the insoluble fibrils of amyloid. Insulin is the hypothesized precursor protein in localized cutaneous insulin-derived (AIns) amyloidosis. Amyloid deposition at insulin injection sites can interfere with absorption, leading to poor glucose control. Despite the increasing prevalence of diabetes mellitus and insulin use, there is a paucity of published cases of AIns amyloidosis and a lack of awareness of this condition among both dermatologists and general practitioners. We report 2 patients with insulin-dependent diabetes who developed hyperkeratotic nodules at insulin injection sites. Our cases complement the current data on iatrogenic amyloidosis and provide insight into this likely underreported phenomenon.

Immunoglobulin light chain amyloidosis presenting as Budd-Chiari syndrome.

Immunoglobulin light chain amyloidosis presenting as Budd-Chiari syndrome.

318: An Atypical Case of COVID-19 and Renal Amyloidosis Presenting With BRASH Syndrome

INTRODUCTION: The syndrome of bradycardia, renal failure, atrioventricular nodal blockade (AVNB), shock and hyperkalemia (BRASH) represents a persistent cycle of pre-existing renal disease and hyperkalemia worsened by bradycardia, hypoperfusion and an AVNB agent We report a continuous cycle of BRASH triggered by COVID-19 that led to diagnosis of renal amyloidosis METHODS: 58-year-old male recently hospitalized for COVID pneumonia with secondary multiorgan failure, requiring temporary mechanical ventilation and continuous renal replacement therapy (CRRT), presented to the emergency department for worsening chills and dyspnea His workup revealed persistent bradycardia (HR 40- 50s bpm), hypotension (MAPs50-70s mmHg), elevated creatinine (2 4 mg/dL), and hyperkalemia (5 2-6 1 mmol/L), thereby raising concern for BRASH Given severity, patient was transferred to ICU for monitoring via arterial line and anticipation of vasopressors, CRRT, and mechanical ventilation His home amiodarone and enalapril were held and hyperkalemia was treated with stabilizing therapies, patiromer and bumetanide Hypotension was addressed with fluids and intermittent albumin Blood pressure, bradycardia, creatinine and hyperkalemia finally improved on third day of admission In the interim, further workup revealed SARS-CoV-2 positive, anasarca and bilateral pleural effusions without evidence of systolic or diastolic dysfunction on echocardiogram He underwent diagnostic and therapeutic thoracentesis that showed transudative fluid, likely sequela of nephrotic syndrome given nephrotic range proteinuria Given M-spike on serum electrophoresis, he underwent kidney biopsy which showed AL amyloid He was discharged to long-term facility for continued diuresis without AVNB or other antihypertensive agents RESULTS: Patient likely had decompensation of preexisting renal amyloidosis due to systemic hypotension from COVID that resulted in a persistent cycle of BRASH While initially critically ill, he became hemodynamically stable by ending the BRASH cycle through appropriate management of hyperkalemia and discontinuation of precipitating medications This case highlights how early recognition and management of BRASH syndrome and its precipitant, such as COVID, can lead to clinical stability in critically ill patients

1287: AL Amyloidosis Presenting as Syncope With Hemorrhagic Shock due to Splenic Rupture

Keller, Daniel1; Stern, Jeffrey2; Mytinger, Andrea2; Anand, Rahul2; Miller, Kristin2 Author Information

Light chain amyloidosis presenting with autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome

Introduction: Light chain (AL) amyloidosis is known to affect multiple organ systems. Although it can involve the liver, biliary channel pathology is rare. Primary biliary cirrhosis or primary sclerosing cholangitis (PSC) with autoimmune hepatitis (AIH) overlap syndrome is a very unusual presentation of systemic amyloidosis and to the best of our knowledge, not reported before. We present a rare case of a 46-years-old female with systemic amyloidosis secondary to AL amyloidosis manifesting primarily as nephrotic syndrome and AIH-PSC overlap syndrome. Patient concerns: In this report, we present a 46-years-old previously healthy female who came with a 1-month history of a right upper quadrant pain and pruritis. She had hypercalcemia, deranged liver enzymes, nephrotic range proteinuria and positive Anti-smooth muscle antibody. Diagnosis: The patient was diagnosed as AIH-PSC overlap syndrome secondary to AL amyloidosis based on clinical symptoms, biochemical, and magnetic resonance cholangiopancreatography findings. She had nephrotic range proteinuria, and upon investigation, kidney biopsy revealed AL amyloidosis. A bone marrow biopsy confirmed the presence of a plasma cell neoplasm. Interventions: The patient received Ursodeoxycholic acid for pruritis. Magnetic resonance cholangiopancreatography was done to diagnose PSC. A kidney biopsy was done to diagnose amyloidosis. Bone marrow biopsy confirmed plasma cell neoplasm, for which she received bortezomib, cyclophosphamide and dexamethasone with a plan of autologous stem cell transplant. Outcomes: The patient was asymptomatic on her follow-up visit after 1 week of discharge and tolerated chemotherapy well. Her liver enzymes (Alkaline phosphatase, Alanine aminotransferase, Aspartate aminotransferase had decreased significantly. Conclusions: AL amyloidosis can very rarely present as AIH-PSC overlap syndrome, manifesting with raised liver enzymes, biliary strictures, pruritis and positive liver antibodies. Patients with amyloidosis involving multiple organ systems, especially at a young age, should be worked up to rule out malignancy. The treatment of AIH-PSC overlap syndrome should be accompanied by that of the primary amyloidosis for definitive management.

S2578 Massive Hepatomegaly on Arrival From Mexico: Keep the Differential Broad

INTRODUCTION: Amyloidosis is a broad term used to denote the extracellular deposition of protein fibrils in various organs, the most common being light chain amyloid (AL) or primary amyloidosis. Amyloidosis can affect any organ system and commonly manifests with organomegaly, cardiomyopathy and renal failure. The diagnosis is made by tissue biopsy staining positive for Congo red. The relative infrequency and wide variations in clinical presentation can make amyloidosis a difficult diagnosis. CASE DESCRIPTION/METHODS: A 52-year-old male who recently immigrated from Mexico, with a past medical history of hypertension, gout and recently diagnosed kidney disease presented with complaints of 20 lb weight loss and generalized malaise over the last three months. He had recently been started on corticosteroids in Mexico, which he was told was for abnormal kidney function. The patient appeared cachectic and exhibited diffuse abdominal tenderness with a palpable abdominal mass and bilateral lower extremity edema. Vital signs were within normal limits. Labs were remarkable for hemoglobin 11.0 g/dL, BUN 117 mg/dL, creatinine 5.27 mg/dL, alkaline phosphatase 305 U/L, GGT 734 U/L, AST 66 U/L and ALT 66 U/L. He also had nephrotic range proteinuria. CT of the abdomen showed hepatomegaly with a 3 × 4 cm mass, likely a hemangioma, which was unable to be confirmed by subsequent MRI, due to lack of contrast. In light of recent immigration, the differential diagnosis was even broader than normal. The patient was started on a diuretic regimen with good response and underwent a bone marrow and kidney biopsy during the admission. Bone marrow biopsy showed a plasma cell neoplasia positive for amyloidosis. Kidney biopsy showed positive Congo red stain with characteristic apple green birefringence under polarized light and positive staining for lambda without kappa. These findings were diagnostic for AL amyloidosis. The patient was later started on a chemotherapy regimen of cyclophosphamide, bortezomib and dexamethasone with plan for autologous bone marrow transplantation. DISCUSSION: We present a rare case of AL amyloidosis presenting with multi-organ failure. A high degree of clinical suspicion is needed in diagnosing amyloidosis, to decrease delays in treatment and avoid life threatening complications like spontaneous hepatic hemorrhage. This case highlights the various presentations of amyloidosis with potential confounders that can delay diagnosis such as a concurrent hepatic mass and recent immigration from Mexico.Figure 1.: Congo red stain of kidney showing amyloid deposition in glomeruli, ×10.Figure 2.: Jones stain showing accumulation of amyloid within the glomeruli, ×20.

S2167 Gastric Amyloidosis Presenting as Acute Upper Gastrointestinal Bleeding

INTRODUCTION: Amyloidosis is characterized by extracellular tissue deposition of fibrils, composed of insoluble low molecular weight protein subunits. The type, location, and extent of fibril deposition generates variable clinical manifestations. Gastrointestinal (GI) amyloid is infrequent and its presentation may be suggestive of more prevalent etiologies. We aim to describe and discuss a case of acute upper GI bleeding secondary to gastrointestinal amyloid. CASE DESCRIPTION/METHODS: A 76-year old male with coronary artery disease, hypertension, and diverticulosis presented to the emergency department with frank hematemesis, melena, and decreased level of consciousness. Management required ICU admission with transfusion, intubation, and hemodynamic support. Upper endoscopy was concerning for the presence of isolated gastric varices, and well as gastritis with erosions in the cardia and antrum. There were no esophageal varices. CT angiography was performed, with embolization of the left gastric artery. This study did not suggest portal hypertension, cirrhosis, or splenic vein thrombosis. “Fullness” seen at the cardia was not secondary to gastric varices. Subsequently, the patient developed bilateral pleural effusions and subsegmental pulmonary embolism with left atrial appendage thrombus, treated by thoracentesis and anticoagulation. Pleural fluid was negative for malignancy. Repeated gastroscopy with biopsies of the cardia were reported as active chronic gastritis and ulceration. However, clinical suspicion for malignancy remained high. DISCUSSION: Pathology was reported as chronic gastritis until case discussion between treating gastroenterologist and pathologist prompted staining for the presence of an infiltrative process. Biopsies from repeat gastroscopy were stained with Congo red, revealing submucosal amyloid deposits. Hematology was consulted and subsequent investigations demonstrated plasma cell dyscrasia and light-chain amyloidosis. Patient was started on protocol for multiple myeloma in January 2020. Unfortunately, the patient developed symptoms of cardiac amyloid, which was strongly suggested by echocardiogram and cardiac markers. There was progressive functional decline and inability to tolerate further chemotherapy. The case illustrates an acute presentation of upper GI amyloid with upper GI bleed. Recognition of the possible pathology is imperative for a timely diagnosis.

S2935 Gastric Mass: A Case of Primary Amyloidosis

INTRODUCTION: We present a rare case of primary amyloidosis presenting as a bleeding gastric mass. Involvement of the gastrointestinal tract is very common in systemic amyloidosis (30-60%), but very rare in primary amyloidosis (8%). CASE DESCRIPTION/METHODS: An 81-year-old man presented to the hospital with anemia with a hemoglobin of 5.3 g/dL. Symptoms on presentation included severe generalized weakness and weight loss. Lab results showed MCV 94.3, Iron 26 ug/dl, Ferritin 15 ng/dl. Upper endoscopy revealed a large ill-defined mass in the gastric body with diffuse ulcerations, friable mucosa, and intramural hematoma (Figure 1). The mass was biopsied, and histology revealed Congo red positive amorphous proteinaceous material consistent with amyloid with apple-green birefringence under polarized light. (Figures 2A and 2B). Serum protein electrophoresis revealed a gamma monoclonal gammopathy. Serum immunofixation showed an IgG kappa and a free light chain lambda. A serum-free light chain assay was not obtained. A bone marrow biopsy revealed plasmacytosis, adjunct flow cytometry showed CD 38 + Plasma cell population with cytoplasmic kappa light chain excess/restriction. These findings where indictive of primary light chain amyloidosis (AL) and excluded the diagnosis of multiple myeloma or systemic amyloidosis (AA). DISCUSSION: Primary amyloidosis is a rare entity. The reported incidence in a population-based study was approximately 1 case per 100,000 population per year. Fatigue and weight loss are the most common clinical manifestations. Hemorrhagic manifestations, congestive heart failure, nephrotic syndrome, malabsorption, and constipation are all relatively common. The diagnosis is made by establishing the presence of amyloid in tissue. Amyloid is amorphous, waxy-appearing, and pink in H&E slides. The Congo red stain is the most used technique for confirming amyloid in a tissue biopsy. characteristic apple-green birefringence under polarized light is seen. Involvement of the gastrointestinal tract is very common in systemic amyloidosis (30–60%), but very rare in primary amyloidosis. The exact prevalence of primary amyloidosis involvement of the stomach is unknown, but the involvement of the upper gastrointestinal tract appears to be present in 8% of all cases. The mainstay treatment is chemotherapy. primary amyloidosis has a poor prognosis when compared to systemic amyloidosis with a predicted median survival of <2 years despite treatment.Figure 1.: Large ill-defined mass in the gastric body with diffuse ulcerations and friable mucosa.Figure 2A.: Congo red positive amorphous proteinaceous material.Figure 2B.: Apple green birefringence under polarized light.

Amyloid Myopathy as an Inclusion Body Myositis Mimic

Introduction: Amyloid myopathy is a rare presentation of systemic amyloidosis. Amyloid myopathy can be initially misdiagnosed as sporadic inclusion body myositis (IBM).&#x0D; Methods: We report 4 cases of amyloid myopathy clinically mimicking inclusion body myositis and initially thought to be phenotypically IBM by neuromuscular experts.&#x0D; Results: Case 1 is an 81-year-old woman who presented with distal arm and proximal leg asymmetric weakness (myopathy pattern 4). Case 2 is a 76-year-old man with primary systemic amyloidosis who presented with myopathy pattern 4 and progressive dysphagia for four years. Case 3 is an 82-year-old man with progressive myopathy pattern 4 weakness and swallowing difficulty. Case 4 is a 62-year-old man with progressive bilateral finger flexor weakness. Muscle biopsies in all 4 cases showed perivascular amyloid deposits&#x0D; Discussion: Amyloid myopathy may be clinically indistinguishable from IBM. Muscle biopsy is of critical importance in the evaluation of patients suspected to have IBM.

Understanding and recognizing cardiac amyloidosis.

Cardiac amyloidosis is an infiltrative abnormality that causes myocardial thickening and dysfunction. Historically, it has been underrecognized as a cause of heart failure and was often misdiagnosed. In the past decade, the cardiology community has improved the understanding of the subtypes of these protein-based infiltrates and how they play a role in heart failure. This article reviews the pathophysiology, presentation, diagnosis, and management of cardiac amyloidosis.

Refractory Heart Failure of Unknown Etiology May Be Cardiac Amyloid If Preceded by Hereditary Neurological Symptoms.

Transthyretin cardiac amyloidosis results from the deposition of transthyretin amyloid fibrils in the myocardium. This happens because of the misfolding of genetically normal (wild type - ATTR) or mutant (hereditary ATTR) transthyretin. The clinical presentation of hereditary ATTR cardiac amyloidosis is dependent on the exact site of the amino acid substitution. The V122I gene mutation is most common among people of African descent and usually manifests with cardiomyopathy. The mutations are transmitted in an autosomal dominant manner with variable penetrance and associated with clinical features occurring most commonly after the age of 40. The symptoms of heart failure (HF) may be preceded by several years of vague neurological symptoms which is more concerning if there is no clear explanation. A high index of suspicion is therefore crucial in ensuring prompt diagnosis and therapy, as this may favorably alter the gloomy prognosis associated with cardiac amyloidosis.

P1503A MULTICENTER STUDY OF THE CLINICAL, LABORATORY CHARACTERISTICS AND POTENTIAL PROGNOSTIC FACTORS IN PATIENTS WITH AA AMYLOIDOSIS ON HEMODIALYSIS

Abstract Background and Aims While, AL amyloidosis is more common in western countries, the most common type of amyloidosis is AA amyloidosis in eastern Mediterranean region including Turkey. Although worse prognosis has been attributed to the AL amyloidosis, AA amyloidosis can also be related to higher mortality under renal replacement therapies. However, there are no sufficient data regarding etiology, clinical presentation and prognostic factors of AA amyloidosis. The objective of our study is to evaluate the clinical, laboratory characteristics and possible predictive factors related to mortality in patients with AA amyloidosis undergoing hemodialysis (HD). Method This multicenter, cross-sectional study was a retrospective analysis of 2100 patients on HD and carried out in 14 selected hemodialysis centers throughout Turkey. Thirty-two patients with biopsy proven AA amyloidosis and thirty-two control patients without AA amyloidosis undergoing HD were included between October 2018 and October 2019. There was no significant difference between the groups in terms of the age and dialysis vintage. Causes of AA amyloidosis, treatment (colchicine and/or anti-interleukin 1 (IL) treatment; canakinumab or anakinra) and number of Familial Mediterranean Fever (FMF) attacks in the last year in case of FMF, systolic and diastolic blood pressures, biochemical values such as mean CRP, hemoglobin, serum albumin, phosphorus, calcium, PTH, ferritin, transferrin saturation, total cholesterol levels and EPO dose, erythropoietin resistance index (ERI), interdialytic fluid intake, body mass indexes, heparin dosage, UF volume and Kt/V data in the last year were collected by retrospective review of medical records. Results Prevalence of AA amyloidosis was found 1.87% in HD centers. In amyloidosis and control groups, 56% and 53% were male, mean age was 54±11 and 53±11 years, mean dialysis vintage was 104±94 and 107±95 months, respectively. FMF was the most common cause of AA amyloidosis (59.5%). All FMF patients received colchicine and the mean colchicine dose was 0,70 ±0,30 mg/day. 26,3 % of FMF patients were unresponsive to colchicine and anti-IL-1 treatment was used in these patients. In AA amyloid and control groups, ERI were 7,88±3,78 and 5,41±3,06 IU/kg/week/g/dl, respectively, (p=0.008). Additionally, higher CRP values (18,78±18,74 and 10,61±10,47 mg/L, p=0.037), lower phosphorus (4,68±0,73 mg/dL vs 5,25±1,04, p=0.014), total cholesterol (135±42 vs 174±39 mg/dL, p&amp;lt;0.01) and serum albumin (3,67±0,49 mg/dL, 4,03±0,22, p&amp;lt;0.01) were observed in patients with AA amyloidosis compared to the control group. Conclusion In this study, we found that long term prognostic factors including higher inflammation, malnutritional parameters and higher ERI were more frequent in AA amyloidosis patients under hemodialysis treatment. Figure Demographic, clinical and laboratory characteristics of patients

MON-469 Rapidly Expanding Thyroid Goiter as the First Manifestation of Systemic Amyloidosis

INTRODUCTIONAmyloidosis is a condition manifesting with extracellular tissue deposition of fibrils of low molecular weight proteins. In Ig light chain amyloidosis (AL), the deposition of Ig light chains can cause organ dysfunction, and patients can have involvement of a range of organs including kidney (70%), liver (70%), heart (60%), peripheral nerves (20%), the tongue, skin, and the coagulation system (1). We describe a unique case of AL amyloidosis, which first presented with thyroid involvement.CLINICAL CASEA 78 year old female patient with hx of small lymphocytic lymphoma and normal thyroid function presented with an expanding symptomatic goiter and compressive symptoms (positive Pemberton sign) for which a total thyroidectomy was performed. Pathology showed Congo red-birefringent amyloid deposition. SPEP showed a small amount of M protein, with circulating monoclonal free lambda light chain on immunofixation. The free kappa lambda light chain ratio was low (0.04) with an elevated serum free lambda light chain (283.93). Mass spectrometry confirmed AL light chain amyloidosis - lambda type. A work up was initiated to assess other organ involvement. Echocardiogram showed mild thickening of left ventricle with a preserved EF and EKG showed low voltage in limb leads. Creatinine (1.05) was minimally elevated from baseline with minimal proteinuria (396 mg/24 hours). Alkaline phosphatase, APTT, and PT were normal. The patient described tongue enlargement and scalloping of the tongue from tooth impingement was seen. Biopsies of tongue and bone marrow also showed amyloidosis thereby securing the diagnosis of systemic amyloidosis, and chemotherapy was initiated.CONCLUSIONThis case illustrates the importance of considering amyloid goiter in the differential for a rapidly enlarging thyroid, even when there is no history of amyloidosis. Although extremely uncommon, a few case reports have described amyloid goiter. In our case, thyroid AL was the initial presentation of systemic amyloidosis. Because disease can be localized or systemic, work up should include an assessment for the presence of a monoclonal gammopathy and an assessment for amyloid mediated organ dysfunction. Tissue should be sent for amyloid typing – there are 35 different proteins known to form amyloid fibrils and prognosis and treatment depends on amyloid type (2). Typing using Liquid Chromatography-Tandem Mass Spectrometry, is the most sensitive and specific methodology, though, in experienced hands, typing by immune histochemistry is an option.REFERENCES1. Rajkumar, S.V., Dispenzieri, A. Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL) amyloidosis. Uptodate. 2019.2. Winter, M., Tholey, A., Kristen, A., Röcken, C. MALDI Mass Spectrometry Imaging: A Novel Tool for the Identification and Classification of Amyloidosis. Proteomics 2017, 17, 1700236.

Management of complications of cardiac amyloidosis: 10 questions and answers.

Amyloidosis is a systemic disorder characterized by extracellular deposition of insoluble fibrils. The most common forms are amyloid light chain and amyloid transthyretin (ATTR) amyloidoses. Cardiac involvement may be found in both these forms, and is an important cause of morbidity and mortality. The clinical presentation of cardiac amyloidosis (CA) may be represented by congestive heart failure (HF), possibly progressing to end-stage HF, as well as atrial fibrillation with possible thromboembolic events, and also conduction disturbances related to amyloid infiltration of conduction fibres. Beyond therapies targeting the blood dyscrasia or the ATTR amyloidogenic cascade, a careful choice of drug therapies, need for device implantation, and possibly treatments for advanced HF is then warranted. In the present review, we try to provide a useful guide to clinicians treating patients with CA by enucleating 10 main questions and answering them based on the evidence available as well as expert opinion and our clinical experience.

Breast Amyloidosis Presenting as Bilateral Breast Masses.

Breast Amyloidosis Presenting as Bilateral Breast Masses.

Amyloidosis presenting as a solitary nasal mass

Amyloidosis is characterized by extracellular deposition of amyloid fibrils. Localized collection of amyloid material in the form of a mass is extremely rare. Mr. M presented with epistaxis and was found to have a friable mass in the right maxillary antrum. Excision was followed by the resolution of symptoms, and histopathology revealed subepithelial collection of amorphous pink material. It was positive for methyl violet and Congo red stain and showed apple-green birefringence. Staining for serum amyloid A and kappa/lambda light chains were negative. Workup for multiple myeloma, positron emission tomography scan for other masses, and screening for end-organ damage was also negative. He continues to be asymptomatic on observation 1-year after presentation. Amyloidosis presenting as a nasal mass has been described in less than five patients so far. Local excision appears to be the best treatment and is associated with a low risk of recurrence on long-term follow-up.

Primary Localized Amyloidosis of the Intestine: A Pathologist Viewpoint.

BackgroundLocalized amyloidosis of the intestine is a rare entity, which can clinically masquerade several conditions such as colitis, polyps, and malignant tumors. This study aims to evaluate the clinicopathological features of this entity.MethodsTo evaluate the clinicopathological features of this entity, a comprehensive search of the literature (1960 to 2019) was done using the following keywords: “amyloidosis” and “small intestine” or “duodenum” or “ileum” or “jejunum” or “colon”. We identified 756 studies about gastrointestinal amyloidosis. Data were examined for 27 studies about localized intestinal amyloidosis. The clinicopathological features were described.ResultsThe age at presentation ranged from 29 to 88 years. The male to female ratio was 3:1. The jejunum and sigmoid colon were the most commonly involved sites. Abdominal pain and intestinal obstruction (small intestine), or rectal bleeding (sigmoid region) were the most common clinical presentations. Colonoscopic findings included wall thickening, mucosal ulcerations (small intestine), and tumor-like masses (colon).ConclusionsThe clinical presentations of localized intestinal amyloidosis depend on the site of the deposition of the amyloid. In most cases, amyloid deposits consisted of light chain protein.

Amyloidosis of submandibular lymph node masquerading as a salivary gland tumor: A rare case report with review of literature

Amyloidosis is a disease characterized by the extracellular deposition of fibrillary proteins causing organ dysfunction. Submandibular lymphadenopathy is a rare clinical presentation of amyloidosis. Majority of the patients progress to develop systemic diseases and usually have AL amyloidosis. Here, we report a case of a 40-year-old male, who presented with a left submandibular swelling for 3 years. The clinical diagnosis was malignant submandibular salivary gland tumor. Systemic examination was unremarkable. Histopathological examination of the excised swelling revealed effacement of lymph node architecture by an eosinophilic Schiff poor material. Positive Congo red stain with apple-green birefringence on polarization confirmed it as amyloidosis. Serum protein electrophoresis was negative for M Band. Immunohistochemistry revealed kappa light-chain restriction in the deposits, characterizing it as localized primary amyloidosis of the submandibular lymph nodes.

Systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy: An autopsy case report

Introduction: Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ. Here we report a case of systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy.

AA Amyloidosis Presenting as Crescentic Glomerulonephritis.

AA Amyloidosis Presenting as Crescentic Glomerulonephritis.