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The Scope of Kidney Affection in Monoclonal Gammopathies at All Levels of Clinical Significance.

Multiple myeloma (MM) is one of the most important clonal malignant plasma cell disorders and renal involvement is associated with poor prognosis. Although there are several reasons for renal impairment in MM, the main cause is the toxic effects of monoclonal proteins. Although cast nephropathy is the best known and unchallenged diagnosis for hematologists and pathologists, the renal effects of monoclonal gammopathy can be various. Monoclonal gammopathy of renal significance was proposed by the International Kidney and Monoclonal Gammopathy Research Group for renal lesions in monoclonal gammop

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… C) Yellow-orange birefringence in glomerulus and vessels under polarized light with Congo red (200 x ). D) Mesangial lambda light chain amyloid deposits with direct immunofluorescence (fluorescein, 400 x ). … Apple-green birefringence of amyloid stained by Congo red. Kidney Int. 2012;82:114. doi: 10.1038/ki.2012.89. 25. Kisilevsky R. Amyloid and amyloidoses: differences, common themes, and practical considerations. Mod Pathol. 1991;4:514–518. 26. …

Advanced sperm selection techniques for assisted reproduction.

BACKGROUND: Assisted reproductive technologies (ART) including in vitro fertilisation (IVF) and intracytoplasmic sperm injection (ICSI), combine gametes to enhance the probability of fertilisation and pregnancy. Advanced sperm selection techniques are increasingly employed in ART, most commonly in cycles utilising ICSI. Advanced sperm selection techniques are proposed to improve the chance that structurally intact and mature sperm with high DNA integrity are selected for fertilisation. Strategies include selection according to surface charge; sperm apoptosis; sperm birefringence; ability to bi…

Peritoneal Amyloid as a Presenting Manifestation of AL Amyloid.

Amyloid is a systemic disease characterized by extracellular deposition of misfolded protein. Gastrointestinal and peritoneal deposition of light chain (AL) amyloid is an under-recognized manifestation of this systemic disease, usually as a late sequela. Here we present a case of recently diagnosed AL peritoneal amyloid that presented in the context of recurrent, acute onset abdominal discomfort and was found to have bowel obstruction complicated by perforation in the setting of AL-mediated gastrointestinal tract infiltration and dysmotility.

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… (C) Polarized light examination of Congo red stained bone marrow biopsy at 400x magnification demonstrates apple green birefringence in vascular wall consistent with amyloid. Fig. 4 Gastric Histopathology. … The definitive method for diagnosis remains tissue biopsy. 23 , 24 The combination of fat pad biopsy and bone marrow biopsy has been shown to have an overall diagnostic sensitivity of 83% in AL amyloid. 36 Congo red staining and typical apple-green birefringence under polarized light remains the gold …

Multiple myeloma presenting with amyloid arthropathy as the first manifestation: Two case reports.

BACKGROUND: Multiple myeloma (MM) can be accompanied by amyloidosis, which occurs in a small number of patients and is characterized by deposition of light chains in the joints, leading to multiple myeloma-associated amyloid arthropathy (MAA). As a rare complication of MM, clinical manifestations of MAA are often similar to those of rheumatoid arthritis, and the two are easily confused. CASE SUMMARY: In recent years, our center treated two patients of MM with amyloid arthropathy as the first manifestation, both of whom presented with polyarthritis. After treatment for MM, both patients achiev

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… Under polarized light, apple green birefringence was observed. On immunofluorescence, k light chain was positive, while lambda light chain was negative (Figure 5C and D ). A clear diagnosis of joint amyloidosis was made, and MAA was considered. … Congo red staining was positive with apple-green birefringence under polarized light. Immunofluorescence was positive for κ and λ light chains. These findings confirmed amyloidosis of the joint tissue. The current treatment of AL amyloidosis is similar to that of MM. …

Recent advances in the diagnosis and management of amyloid cardiomyopathy.

Amyloidosis is a disorder characterized by misfolded precursor proteins that form depositions of fibrillar aggregates with an abnormal cross-beta-sheet conformation, known as amyloid, in the extracellular space of several tissues. Although there are more than 30 known amyloidogenic proteins, both hereditary and non-hereditary, cardiac amyloidosis (CA) typically arises from either misfolded transthyretin (ATTR amyloidosis) or immunoglobulin light-chain aggregation (AL amyloidosis). Its prevalence is more common than previously thought, especially among patients with heart failure and preserved

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… Although birefringence under polarized light microscopy is considered histopathologically diagnostic for amyloidosis, Congo Red staining of the biopsy tissue alone is sometimes not sufficient and other methods need to be used 45 . … In particular, Congo Red staining requires a skilled and experienced pathologist who may not be available in all institutions to accurately visualize the apple-green birefringence under polarized light microscopy. …

Amyloidoses in Onco-Nephrology Practice: A Multidisciplinary Case-Based Conference Report.

Introduction and objectiveAmyloidoses are a heterogeneous group of disorders resulting from deposition of amyloid fibrils into extracellular tissues. While the kidneys are one of the most frequent sites of amyloid deposition, amyloid deposits can also affect a wide range of organ systems, including the heart, liver, gastrointestinal tract, and peripheral nerves. The prognosis of amyloidosis, especially with cardiac involvement, remains poor; however, a collaborative approach applying new tools for diagnosis and management may improve outcomes. In September 2021, the Canadian Onco-Nephrology In

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… Biopsied tissue was Congo red–positive with apple-green birefringence on polarized light microscopy, confirming amyloidosis. Skeletal survey ruled out myeloma-related bone lesions. Mass spectrometry confirmed the AL amyloidosis subtype. … Diagnostic focus and assessment: Kidney biopsy revealed nodular lesions and Congo red positivity with apple-green birefringence. Immunofluorescence was negative for kappa and lambda light chain deposition but was positive for Protein A. Electron micrograph revealed the presence of amyloid fibrils. …

Uncovering the knowledge about systemic amyloidosis relevant to the rheumatologists.

Amyloidosis is a localized or systemic disease caused by deposition of proteins in the extracellular space of various organs and tissues. As part of the disease, proteins that were originally soluble misfold and acquire a fibrillar conformation that renders them insoluble and resistant to proteolysis. Systemic amyloidosis is a rare, often underdiagnosed condition. In recent years, the incidence of newly diagnosed cases of amyloidosis has been increasing in association with the aging of the population and greater access to diagnostic tests. From a clinical perspective, systemic amyloidosis is f

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… immunoglobulin fragments, and aging. [ 2 – 4 ] More than 40 types of proteins with the potential to become amyloid are currently recognized, some causing systemic amyloidosis and others causing only localized forms of the disease. [ 5 ] On histopathology, amyloid fibrils stain with Congo red and appear birefringent … In both conditions, a key diagnostic step still involves confirmation through tissue biopsy, typically from an easily accessible site such as abdominal fat, the rectum, or the affected organ, with Congo red staining demonstrating apple-green birefringence under polarized light, indicative of amyloid …

Amyloidosis in Childhood: A Review of Clinical Features and Comparison with Adult Forms.

Amyloidosis is a rare multisystem disorder characterized by extracellular accumulation of insoluble fibrils in various organs and tissues. The most common subtype in the pediatric population is systemic reactive amyloidosis, typically developing secondary to chronic inflammatory conditions and resulting in deposition of serum amyloid A protein in association with apolipoprotein HDL3. Clinical presentation is highly variable and is mostly influenced by specific organs involved, precursor protein type, and extent of amyloid deposition, often closely reflecting clinical features of the underlying

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… These fibrils (around 10 nm in diameter) are constituted of peptides arranged in an antiparallel β-sheet structure, conferring insolubility, resistance to proteolysis, and specific dyeing properties, such as Congo red affinity and apple-green birefringence under polarized light microscopy, in addition … Congo red staining, viewed under polarized light, is a reference standard, revealing characteristic apple-green birefringence due to alternating hydrophobic and hydrophilic regions along the fiber axis [ 57 , 79 ]. …

Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia.

Pulmonary hypertension (PH) has been described in myeloproliferative disorders; monoclonal plasma cell disorder such as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome; and plasma cell dyscrasias such as multiple myeloma and amyloidosis. We describe 4 cases of PH likely due to pulmonary vascular involvement and myocardial deposition from light chain deposition disease, amyloidosis, and multiple myeloma. On the basis of our clinical experience and literature review, we propose screening for plasma cell dyscrasia in patients with heart failure with

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… B, Under polarized light, apple green birefringence is seen (arrows). Figure 3 A, Congo red–stained section reveals amorphous material consistent with amyloid in the myocardial interstitium (arrows). B, Under polarized light, apple green birefringence is seen (arrows). …

Diagnosis and treatment of cystic lung disease.

Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities

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… Apple-green birefringence in tissues stained with Congo red is important for differentiating pulmonary amyloidosis from LCDD [ 9 , 19 ]. … The typical apple-green birefringence on Congo red staining originates from the fibrillary deposits in the lung. There is currently no established curative therapy [ 18 ]. CONCLUSIONS With the increased use of CT, the recognition of CLD has also increased. …

Endoscopic and Histological Diagnosis of Gastrointestinal Amyloidosis: A Narrative Review.

Amyloidosis encompasses a diverse group of systemic and localised disorders defined by the extracellular accumulation of misfolded proteins, leading to progressive tissue damage and multiorgan dysfunction. Gastrointestinal (GI) involvement is common, yet diagnosis is frequently delayed because symptoms are non-specific and endoscopic findings are equivocal. Recent advances in endoscopic technique, biopsy site selection and advancements in processing of pathological specimens have substantially improved the diagnosis of this condition. Targeted gastrointestinal biopsy, performed during endoscop

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… Deposits appear red-orange on conventional light microscopy and exhibit characteristic apple-green birefringence under polarised light (Figure 7 ) [ 34 , 35 ]. The technique confirms the presence of amyloid material; however, it cannot differentiate between different amyloid precursor proteins. … Under polarised light, the amyloid exhibits characteristic "apple-green" birefringence (B, white arrows). This is an original image created by the authors. …

Pulmonary amyloidosis: clinicopathologic spectrum and diagnostic pitfalls in a 12-patient case series.

BACKGROUND: Pulmonary amyloidosis is a rare, underrecognized manifestation of amyloid disease that may present as nodular, tracheobronchial, or diffuse alveolar-septal involvement and can mimic common pulmonary conditions. This study aimed to characterize the clinicopathologic features, diagnostic patterns, and management approaches of patients with pulmonary amyloidosis in a real-world setting. METHODS: We performed a retrospective review of patients with pathology-confirmed pulmonary amyloidosis diagnosed between January 2020 and December 2024 at a tertiary academic medical center in Detroi

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… Participants Patients were eligible for inclusion if they had histopathologic confirmation of pulmonary amyloidosis, defined by Congo red-positive amyloid deposition with apple-green birefringence under polarized light on lung or tracheobronchial tissue specimens. … Congo red staining was performed in all cases (n=12) as part of the diagnostic workup and demonstrated apple-green birefringence under polarized light. These findings are consistent with a diagnosis of pulmonary amyloidosis ( Figure 5 ). …

ATTR Cardiomyopathy Meets Multiple Myeloma: The Importance of Cardiac Biopsy.

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… Ultimately, CA was confirmed because the biopsy demonstrated amyloid deposits on Congo Red stain and fluorescence microscopy ( Figure 1 ), as well as apple-green birefringence by polarized light microscopy. …

Neck swelling in a patient with gout - What is the cause?

We describe the case of a 55-year-old man with underlying tophaceous gout who presented with a 6-month history of multiple joint pains and associated constitutional symptoms. Physical examination revealed firm and non-tender swelling measuring 3x4 cm adjacent to the left clavicular head. This swelling did not move with deglutition. Neck imaging showed bilateral heterogeneous soft tissue lesions at the sternoclavicular, first costochondral and acromioclavicular joints. Multiple lytic lesions with cortical destruction involving the upper manubrium and distal ends of both clavicles were seen. Fin

Endoscopic and pathological characteristics of gastrointestinal amyloidosis: a retrospective analysis.

BackgroundGastrointestinal amyloidosis (GIA) is a rare manifestation of amyloidosis, characterized by amyloid fibril deposition in the gastrointestinal tract, leading to a range of clinical symptoms. Early diagnosis is challenging due to the nonspecific nature of endoscopic and clinical findings.ObjectiveTo analyze the clinical, endoscopic, and pathological characteristics of GIA and identify potential diagnostic markers for earlier detection.MethodsA retrospective study was conducted on 36 patients diagnosed with GIA based on histopathological findings, including Congo Red staining. Clinical,

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… These fibrils are composed of protein fragments that adopt a distinctive β-pleated sheet conformation, and when stained with Congo Red dye, they display green birefringence under cross-polarized light, a hallmark of amyloid deposits [ 2 ]. … This finding was further confirmed through Congo Red staining, as shown in Fig. 1 , with green birefringence observed under polarized light microscopy (Fig. 2 ). Fig. 1 Endoscopic images and corresponding histopathological images of gastrointestinal amyloidosis at various sites. …

Редкое дерматологическое проявление фолликулярных смешений у пациента с множественной миеломой и конечной стадией почечной болезни при гемодиализе: отчет по делу.

Множественная миелома (ММ) — гематологическая порча, характеризующаяся клональным расширением злокачественных плазменных клеток в костном мозге, что приводит к различным системным осложнениям. Хотя резкие проявления MM редки, фолликулы представляют собой весьма специфический, но редкий дерматологический вывод, связанный с MM. В настоящем исследовании представлен пример 59-year-old Человек с MM, который демонстрировал фолликулярный акантоз и гиперкератоз в качестве скромных индикаторов злодеяний. Пациент с историей сцены 5 В ходе клинической оценки и лабораторных исследований был диагностирован

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… Under polarized light, these deposits exhibited classic apple-green birefringence, confirming amyloid deposition ( Fig. 3A ). Figure 3 Immunohistochemical characterization of bone marrow biopsy. … (A) Congo red staining showed amyloid deposits with birefringence under polarized light, confirming amyloidosis. (B) Scattered CD3 + T cells. (C) CD20 + B cells. (D) Diffuse CD56 positivity indicating natural killer cell involvement. (E) Diffuse CD138 staining marking plasma cell infiltration. …

Immunoglobulin Light-Chain Amyloidosis: Clinical Presentations and Diagnostic Approach.

Systemic immunoglobulin light-chain (AL) amyloidosis is a rare disorder arising from a plasma cell clone that produces misfolded immunoglobulin light chains, which are deposited in various tissues and organs as amyloid fibrils. Signs and symptoms are typically vague and overlap with those arising from other common diseases; consequently, diagnosis of AL amyloidosis is challenging for clinicians. Substantial delays between onset of symptoms and diagnosis are common, and result in poorer outcomes, particularly in patients with cardiac AL amyloidosis and others who develop advanced organ dysfunct

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… When stained using Congo red, the amyloid deposits produce a pathognomonic red-green birefringence under cross-polarized light microscopy ( Comenzo, 2007a ; Falk et al., 1997 ; Merlini & Bellotti, 2003 ). … However, Congo red staining and fluorescent birefringence alone do not distinguish AL amyloidogenic protein from other forms of amyloidosis such as transthyretin (TTR), hereditary apolipoprotein AI, or acquired amyloid A (AA) amyloidosis due to chronic inflammation; typing of the amyloid deposits is …

Hereditary transthyretin amyloidosis presenting with carpal tunnel syndrome.

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… pathogenic variants. 9 The gold standard for diagnosing hereditary transthyretin amyloidosis is a biopsy, preferably from abdominal subcutaneous fatty tissue; 2 sites are recommended because of the patchy distribution of amyloid deposits. 2 , 6 , 8 Congo red staining shows amyloid fibrils with apple-green birefringence …

Clinical effects of tranexamic acid on bleeding tendency due to fibrinolytic activation of AL amyloidosis.

We report a case where tranexamic acid, which is an antifibrinolytic agent, was used to effectively treat bleeding tendency in a patient with immunoglobulin light chain (AL) amyloidosis. A male patient in his 80s without a history of bleeding disorders was admitted to our hospital for the examination of bleeding tendency and was diagnosed with a bleeding disorder due to AL amyloidosis. Blood tests revealed elevated plasmin-α2-plasmin inhibitor complex levels, suggesting fibrinolytic activation. Managing the bleeding was difficult; however, we suspected fibrinolytic activation associated with A

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… Figure 3 Pathology of the duodenal biopsy duodenal mucosa with apple green birefringence under polarised light microscopy. Left, H&E staining: ×100; right, congo red staining: ×400. …

AL Amyloidosis: Unfolding a Complex Disease.

Light chain (AL) amyloidosis is a rare plasma cell dyscrasia. An estimated 12,000 people live with the disease in the United States. AL amyloidosis occurs from the misfolding of proteins that deposit in organs (heart, kidneys, digestive tract, tongue, lungs, and nervous system), leading to progressive organ damage and impairment of quality of life. The treatment of AL amyloidosis has improved greatly over the past several years, with new treatments currently in development. This article will focus on the pathophysiology, diagnosis, and treatment of AL amyloidosis.

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… When viewed under cross-polarized light, this reveals apple-green birefringence, a characteristic that is the histologic gold standard for detecting amyloid ( Wechalekar et al., 2015 ; see Figure 1 ). Additionally, the patient should undergo a bone marrow biopsy with Congo red staining. … (C) Apple green birefringent materials in the Congo red–stained soft tissue viewed under polarized light. While these techniques have a high sensitivity and specificity in diagnosing amyloidosis, the gold standard for diagnosis and typing is mass spectrometry of amyloid deposits. …